版本
5.0
创建日期
2005-11-16 15:48:42 UTC
更新日期
2021-09-14 15:46:27 UTC
登录号
HMDB0000062
地位
quantified
二级访问
HMDB0001467

HMDB00062

HMDB01467

名称
L-Carnitine
描述
Carnitine is a non-essential amino acid and a quaternary ammonium compound. Carnitine is also classified as an alcohol (specifically, a trimethylated carboxy-alcohol). Carnitine exists as one of two stereoisomers (the two enantiomers D-carnitine and L-carnitine. Both are biologically active, but only L-carnitine naturally occurs in animals, and D-carnitine is toxic as it inhibits the activity of the L-form. Carnitine is involved in the metabolism in most mammals, plants, and some bacteria. Carnitine plays a key role in lipid metabolism and beta-oxidation. It is used to transport long-chain fatty acids into the mitochondria to be oxidized for energy production. This is done by forming a long chain acetylcarnitine esters which are then transported by carnitine palmitoyltransferase I and carnitine palmitoyltransferase II. Carnitine also participates in removing products of metabolism from cells. Given its key metabolic roles, carnitine is concentrated in skeletal and cardiac muscle as well as other tissues that metabolize fatty acids as an energy source. A normal 70 kilogram person typically produces 11–34 mg of carnitine per day. Adults eating mixed diets of red meat and other animal products ingest 60–180 mg of carnitine per day, while vegans consume about 10–12 mg per day. Most carnitine obtained from the diet is absorbed in the small intestine before entering the blood.[3] The total body content of carnitine is about 20 grams in a person weighing 70 kilograms, with nearly all of it contained within skeletal muscle cells. Carnitine is so important in providing energy to muscles (including the heart) that some researchers are now recommending carnitine supplements in the diet, particularly for people who do not consume much red meat (the main food source for carnitine). Carnitine has been described as a vitamin, an amino acid, or a metabimin (i.e. an essential metabolite). Like the B vitamins, carnitine contains nitrogen and is very soluble in water. However, most animals, including humans, make their own carnitine; thus, carnitine cannot be considered to be a vitamin. In certain circumstances, such as methionine deficiency, lysine deficiency, vitamin C deficiency or kidney dialysis, carnitine shortages can develop. Under these conditions, carnitine must be absorbed from food, and for this reason, it is sometimes referred to as a "metabimin" or a conditionally essential metabolite. In humans, about 25% of carnitine is synthesized in the liver, kidney, and brain from lysine and methionine. Most of the carnitine in the body comes from dietary sources such as red meat and dairy products. Inborn errors of carnitine metabolism such as Reye’s syndrome can lead to brain deterioration gradually worsening muscle weakness, Duchenne-like muscular dystrophy, and extreme muscle weakness with fat accumulation in muscles. Carnitine is an essential nutrient for pre-term babies and individuals who are unable to eat a normal diet (e.g. non-ketotic hypoglycemics, kidney dialysis patients) (PMID: 115309). In conditions such as kwashiorkor, cirrhosis, and heart muscle disease (cardiomyopathy) as well as in inborn errors of metabolism such as type IV hyperlipidemia and propionic aciduria, carnitine is essential to life and carnitine supplements are critically important. Carnitine therapy may also be useful in a wide variety of clinical conditions. Carnitine supplementation has improved some patients who have angina secondary to coronary artery disease. Carnitine supplements may also be useful in many forms of metabolic liver diseases and heart muscle disease. Hearts undergoing severe arrhythmia quickly deplete their stores of carnitine. Athletes, particularly in Europe, have used carnitine supplements for improved endurance. Carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. Carnitine may be of value in treating pregnant women, hypothyroid individuals, and male infertility due to the low motility of sperm. Carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate muscular weakness with associated anorexia. Carnitine is a biomarker for the consumption of meat. In fact, carnitine was first extracted from meat extracts in 1905, leading to its name from Latin, "caro/carnis" or flesh.
同义词
1:(-)-Carnitine

2:(-)-L-Carnitine

3:3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium hydroxide, inner salt

4:Carnicor

5:Carnitene

6:Carnitine

7:Carnitor

8:Karnitin

9:Levocarnitine

10:Vitamin BT

11:L-gamma-Trimethyl-beta-hydroxybutyrobetaine

12:(R)-Carnitine

13:L-g-Trimethyl-b-hydroxybutyrobetaine

14:L-Γ-trimethyl-β-hydroxybutyrobetaine

15:(-)-(R)-3-Hydroxy-4-(trimethylammonio)butyrate

16:(R)-(3-Carboxy-2-hydroxypropyl)trimethylammonium hydroxide

17:(S)-Carnitine

18:1-Carnitine

19:3-Carboxy-2-hydroxy-N,N,N-trimethyl-1-propanaminium

20:3-Hydroxy-4-trimethylammoniobutanoate

21:3-Hydroxy-4-trimethylammoniobutanoic acid

22:Bicarnesine

23:Carniking

24:Carniking 50

25:Carnilean

26:Carnipass

27:Carnipass 20

28:D-Carnitine

29:delta-Carnitine

30:DL-Carnitine

31:gamma-Trimethyl-ammonium-beta-hydroxybutirate

32:gamma-Trimethyl-beta-hydroxybutyrobetaine

33:gamma-Trimethyl-hydroxybutyrobetaine

34:L-(-)-Carnitine

35:Levocarnitina

36:Levocarnitinum

37:R-(-)-3-Hydroxy-4-trimethylaminobutyrate

38:L Carnitine

39:L-Carnitine

化学式
C7H16NO3
平均分子量
162.2068
等位分子量
162.113018383
化学名称
(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate
传统名称
L-carnitine
CAS 登记号
541-15-1
代谢物结构字符串
C[N+](C)(C)C[C@H](O)CC(O)=O
inchi标识符
InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/p+1/t6-/m1/s1
印记键
PHIQHXFUZVPYII-ZCFIWIBFSA-O
分类学
description: belongs to the class of organic compounds known as carnitines. These are organic compounds containing the quaternary ammonium compound carnitine.

description:Carnitines

kingdom:Organic compounds

super_class:Organic nitrogen compounds

class:Organonitrogen compounds

sub_class:Quaternary ammonium salts

molecular_framework:Aliphatic acyclic compounds

1:1,2-aminoalcohols

2:Beta hydroxy acids and derivatives

3:Carbonyl compounds

4:Carboxylic acid salts

5:Carboxylic acids

6:Fatty acids and conjugates

7:Hydrocarbon derivatives

8:Monocarboxylic acids and derivatives

9:Organic oxides

10:Organic salts

11:Organic zwitterions

12:Organopnictogen compounds

13:Secondary alcohols

14:Short-chain hydroxy acids and derivatives

15:Tetraalkylammonium salts

16:1,2-aminoalcohol

17:Alcohol

18:Aliphatic acyclic compound

19:Amine

20:Beta-hydroxy acid

21:Carbonyl group

22:Carboxylic acid

23:Carboxylic acid derivative

24:Carboxylic acid salt

25:Carnitine

26:Fatty acid

27:Hydrocarbon derivative

28:Hydroxy acid

29:Monocarboxylic acid or derivatives

30:Organic oxide

31:Organic oxygen compound

32:Organic salt

33:Organic zwitterion

34:Organooxygen compound

35:Organopnictogen compound

36:Secondary alcohol

37:Short-chain hydroxy acid

38:Tetraalkylammonium salt

39:carnitine

本体论
term:Physiological effect

definition:The effect on an organism physiology, resulting from its exposure to a chemical.

parent_id:

level:1

type:parent

term:Health effect

definition:A health condition or observation associated with a stimuli or with a biological activity of a chemical.

parent_id:7693

*level:2

type:parent

term:Observation

definition:A short term health effect which reflects the reponse of the body to stimuli or is a symptom of a disease or condition; also includes health status which reflects stage in life.

parent_id:7694

**level:3

type:parent

term:Tachycardia

definition:

parent_id:7717

***level:4

type:child

synonym:Increased heart rate

term:Fever

definition:

parent_id:7717

***level:4

type:child

term:Attachment loss

definition:

parent_id:7717

***level:4

type:child

term:Missing teeth

definition:

parent_id:7717

***level:4

type:child

term:Periodontal Probing Depth

definition:

parent_id:7717

***level:4

type:child

term:Tooth Decay

definition:

parent_id:7717

***level:4

type:child

term:Health condition

definition:A health effect that consists on short or long-term disease, condition, disorder, syndrome or constant abnormality.

parent_id:7694

**level:3

type:parent

term:Diabetes mellitus type 2

definition:

parent_id:7695

***level:4

type:child

synonym:Non-insulin-dependent diabetes mellitus

synonym:Niddm

synonym:Adult-onset diabetes

term:Nervous system disorders

definition:Any disorder (non-neoplastic or neoplastic) that affects the brain, spinal cord, or peripheral nerves.

parent_id:7695

***level:4

type:parent

term:Vascular disorders

definition:Any disorder that affects the blood vessels.

parent_id:7695

***level:4

type:parent

term:Cancer

definition:A group of disorders characterized by the presense of malignant neoplasm.

parent_id:7695

***level:4

type:parent

term:Disposition

definition:A concept that describes the origin of a chemical, its location within an organism, or its route of exposure.

parent_id:

level:1

type:parent

term:Route of exposure

definition:A mean by which a chemical agent comes in contact with an organism, either under intended or unintended circumstances.

parent_id:7724

*level:2

type:parent

term:Enteral

definition:Chemical exposure via the alimentary canal (mouth to anus).

parent_id:7743

**level:3

type:parent

term:Ingestion

definition:Chemical exposure facilitated by entry through the mouth.

parent_id:7744

***level:4

type:child

synonym:Digestion

term:Parenteral

definition:Chemical exposure via the a route other than the alimentary canal.

parent_id:7743

**level:3

type:parent

term:Intravenous

definition:Chemical exposure via direct introduction into the circulatory system a the vein.

parent_id:7745

***level:4

type:child

synonym:Irrigation

synonym:Intra-venous

term:Source

definition:Natural or synthetic origin of a chemical.

parent_id:7724

*level:2

type:parent

term:Endogenous

definition:

parent_id:7735

**level:3

type:child

term:Food

definition:

parent_id:7735

**level:3

type:child

term:Synthetic

definition:A man-made product or sample in which a chemical can be found.

parent_id:7735

**level:3

type:parent

term:Personal care product

definition:

parent_id:7742

***level:4

type:child

synonym:Pcp

synonym:Toiletries

synonym:Toiletry

synonym:Personal hygiene

synonym:Beautification product

term:Biological

definition:A living organism (species or a higher taxonomy rank), in which a chemical can be found.

parent_id:7735

**level:3

type:parent

term:Animal

definition:A living organism belonging to the kingdom animalia. it feeds on organic matter, typically having specialized sense organs and nervous system and able to respond rapidly to stimuli.

parent_id:7736

***level:4

type:child

synonym:Fauna

term:Plant

definition:A living organism belonging to the kingdom plantea. typically, it grows in a permanent site, absorbs water and inorganic substances through its roots, and synthesizes nutrients in its leaves by photosynthesis using the green pigment chlorophyll. examples incude trees, shrubs, herbs, grasses, ferns, and mosses.

parent_id:7736

***level:4

type:parent

synonym:Flora

term:Biological location

definition:The physiological origin within an organism, including anatomical compnents, biofluids and excreta.

parent_id:7724

*level:2

type:parent

term:Organ and components

definition:An anatomical organizational level including multiple tissues or substructures, which enables a common biological function.

parent_id:7725

**level:3

type:parent

term:Intestine

definition:The portion of the gastrointestinal tract that includes the small and large intestines.

parent_id:7727

***level:4

type:child

term:Testicle

definition:Either of the paired male reproductive glands that produce the male germ cells and the male hormones. (nci).

parent_id:7727

***level:4

type:child

synonym:Testes

synonym:Testis

term:Bladder

definition:The distensible sac-like organ that functions as a reservoir of urine, collecting from the kidneys and eliminating via the urethra. (nci).

parent_id:7727

***level:4

type:child

term:Brain

definition:An organ composed of grey and white matter that is the center for intelligence and reasoning. it is protected by the bony cranium. (nci).

parent_id:7727

***level:4

type:child

term:Lung

definition:A thoracic organ that has variable lobation and is the primary respiratory organ of mammals.

parent_id:7727

***level:4

type:child

synonym:Lungs

term:Liver

definition:An abdominal organ that has variable lobation which are composed mainly of hepatic lobules. (nci).

parent_id:7727

***level:4

type:child

term:Prostate

definition:The male reproductive accessory gland that produces prostatic fluid and is located adjacent to or around the urethra distal to the urinary bladder in mammals. (nci).

parent_id:7727

***level:4

type:child

synonym:Prostate gland

term:Kidney

definition:The organs of the urinary tract located in the retroperitoneal cavity adjacent to the spine and composed of the renal cortex and the renal medulla. (nci).

parent_id:7727

***level:4

type:child

synonym:Kidneys

term:Tissue and substructures

definition:An anatomical organizational level including multiple cells yet not comprising a complete organ .

parent_id:7725

**level:3

type:parent

term:Skeletal muscle

definition:Striated muscles that are under voluntary control of the organism. they are connected at either or both ends to a bone and are utilized for locomotion and other movements. (nci).

parent_id:7729

***level:4

type:child

synonym:Striated muscle

term:Adipose tissue

definition:A specialized form of connective tissue consisting primarily of adipocytes (fat cells), surrounded by a meshwork of collagen fibers. (NCI)

parent_id:7729

***level:4

type:child

synonym:Fat tissue

synonym:Body fat

synonym:Adipose

term:Cardiac muscle

definition:The striated muscle tissue of the heart enveloped by the epicardium and the endocardium.

parent_id:7729

***level:4

type:child

synonym:Myocardium

synonym:Myocardial tissue

synonym:Heart muscle

term:Muscle

definition:A fibrous soft tissue with the ability to contract to produce force and motion. (nci).

parent_id:7729

***level:4

type:child

term:Placenta

definition:An organ present in some vertebrates during embryonic gestation that surrounds the fetus and provides it with nutrients and oxygen, facilitates gas and waste exchange between the fetus and mother, and provides parasitic cloaking from the mother's immune system by excretion of neurokinin b. (nci).

parent_id:7729

***level:4

type:child

term:Biofluid and excreta

definition:A liquid, semi-solid or solid material originating in the body.

parent_id:7725

**level:3

type:parent

term:Urine

definition:Excretion in liquid state processed by the kidney.

parent_id:7731

***level:4

type:child

term:Saliva

definition:The watery fluid in the mouth made by the salivary glands. saliva moistens food to help digestion and it helps protect the mouth against infections. (nci).

parent_id:7731

***level:4

type:child

term:Feces

definition:The material discharged from the bowel during defecation. It consists of undigested food, intestinal mucus, epithelial cells, and bacteria.

parent_id:7731

***level:4

type:child

synonym:Fecal

synonym:Stool

synonym:Faecal

synonym:Faeces

term:Blood

definition:A liquid tissue with the primary function of transporting oxygen and carbon dioxide (nci). it supplies the tissues with nutrients, removes waste products, and contains various components of the immune system defending the body against infection.

parent_id:7731

***level:4

type:child

term:Breast milk

definition:Portion of secreted substance produced by the mammary gland.

parent_id:7731

***level:4

type:child

term:Cerebrospinal fluid

definition:The fluid that is contained within the brain ventricles, the subarachnoid space and the central canal of the spinal cord. (nci).

parent_id:7731

***level:4

type:child

synonym:Csf

term:Subcellular

definition:An anatomical organizational level including a component within a biological cell .

parent_id:7725

**level:3

type:parent

term:Cytoplasm

definition:The portion of the cell contained within the plasma membrane but excluding the nucleus.

parent_id:7730

***level:4

type:child

synonym:Cytoplasma

term:Mitochondria

definition:

parent_id:7730

***level:4

type:child

term:Endoplasmic reticulum

definition:A network of tubular membranes within the cytoplasm of the cell, occurring either with a smooth surface (smooth endoplasmic reticulum) or studded with ribosomes (rough endoplasmic reticulum), involved in the transport of materials. (Infoplease Dictionary via NCI)

parent_id:7730

***level:4

type:child

synonym:Er

term:Peroxisome

definition:A small organelle enclosed by a single membrane, and found in most eukaryotic cells. Contains peroxidases and other enzymes involved in a variety of metabolic processes including free radical detoxification, lipid catabolism and biosynthesis, and hydrogen peroxide metabolism. (Gene Ontology).

parent_id:7730

***level:4

type:child

synonym:Peroxisome vesicle

synonym:Peroxisomal

term:Cell and elements

definition:An anatomical organizational level comprising of a single cell (or a fragment in some exceptions). a cell is defined by its type or function. it is the smallest unit of living structure capable of independent existence, composed of a membrane-enclosed mass of protoplasm and containing a nucleus or nucleoid.

parent_id:7725

**level:3

type:parent

term:Extracellular

definition:The space external to the outermost structure of a cell.

parent_id:7732

***level:4

type:child

synonym:Extracellular region

term:Element

definition:

parent_id:7732

***level:4

type:parent

term:Cell

definition:

parent_id:7732

***level:4

type:parent

term:Process

definition:Biological or chemical events, or a series thereof, leading to a known function or end-product.

parent_id:

level:1

type:parent

term:Naturally occurring process

definition:Naturally-occurring molecular events or a series thereof, leading to a known function or end-product.

parent_id:7659

*level:2

type:parent

term:Biological process

definition:Biological or chemical events or a series thereof, leading to a known function or end-product within an organism.

parent_id:7660

**level:3

type:parent

term:Biochemical pathway

definition:A linked series of chemical reactions that occur in a defined order within or between organism cells, and lead to a known function or end product.

parent_id:7661

***level:4

type:parent

term:Biochemical process

definition:A biological process occurring at the subcellular level, involving biochemical reactions.

parent_id:7661

***level:4

type:parent

term:Role

definition:The purpose or function assumed by a chemical, either naturally or as intended by humans .

parent_id:

level:1

type:parent

term:Industrial application

definition:The assumed function of a chemical utilized by human.

parent_id:7671

*level:2

type:parent

term:Personal care products

definition:Cosmetics and personal hygene products in which a chemical was incorportaed during the manufacturing process.

parent_id:7678

**level:3

type:child

term:Food and nutrition

definition:A chemical purpose related to manufacturing of food and nutrition products.

parent_id:7678

**level:3

type:parent

term:Nutritional supplement

definition:

parent_id:7687

***level:4

type:child

synonym:Nutraceutical

synonym:Dietary supplement

term:Pharmaceutical industry

definition:A pharmacologic activity for which a chemical substance is utilized owing to its biological role.

parent_id:7678

**level:3

type:parent

term:Pharmaceutical

definition:

parent_id:7679

***level:4

type:child

term:Biological role

definition:The biological function of a chemical. the biological role answers the question how a chemical is involved in molecular processes in an organism. this can include biochemical effects of non-endogenous chemicals, which are also assigned an industrial application such as pharmaceuticals. the biological role is limited to cellular levls, and will not include role at system process level, such as a chemical which has a role in a disease.

parent_id:7671

*level:2

type:parent

term:Essential amino acid

definition:

parent_id:7672

**level:3

type:child

term:Vitamin

definition:

parent_id:7672

**level:3

type:child

状态
Solid
实验性质
1:

kind:melting_point

value:197 °C

source:

预测性质
1:

kind:logp

value:-2.90

source:ALOGPS

2:

kind:logs

value:-1.60

source:ALOGPS

3:

kind:solubility

value:5.33 g/L

source:ALOGPS

4:

kind:logp

value:-4.9

source:ChemAxon

5:

kind:pka_strongest_acidic

value:4.2

source:ChemAxon

6:

kind:pka_strongest_basic

value:-3.6

source:ChemAxon

7:

kind:iupac

value:(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate

source:ChemAxon

8:

kind:average_mass

value:162.2068

source:ChemAxon

9:

kind:mono_mass

value:162.113018383

source:ChemAxon

10:

kind:smiles

value:C[N+](C)(C)C[C@H](O)CC(O)=O

source:ChemAxon

11:

kind:formula

value:C7H16NO3

source:ChemAxon

12:

kind:inchi

value:InChI=1S/C7H15NO3/c1-8(2,3)5-6(9)4-7(10)11/h6,9H,4-5H2,1-3H3/p+1/t6-/m1/s1

source:ChemAxon

13:

kind:inchikey

value:PHIQHXFUZVPYII-ZCFIWIBFSA-O

source:ChemAxon

14:

kind:polar_surface_area

value:60.36

source:ChemAxon

15:

kind:refractivity

value:63.49

source:ChemAxon

16:

kind:polarizability

value:16.93

source:ChemAxon

17:

kind:rotatable_bond_count

value:4

source:ChemAxon

18:

kind:acceptor_count

value:3

source:ChemAxon

19:

kind:donor_count

value:1

source:ChemAxon

20:

kind:physiological_charge

value:0

source:ChemAxon

21:

kind:formal_charge

value:0

source:ChemAxon

22:

kind:number_of_rings

value:0

source:ChemAxon

23:

kind:bioavailability

value:Yes

source:ChemAxon

24:

kind:rule_of_five

value:Yes

source:ChemAxon

25:

kind:ghose_filter

value:No

source:ChemAxon

26:

kind:veber_rule

value:No

source:ChemAxon

27:

kind:mddr_like_rule

value:No

source:ChemAxon

1:

type:Specdb::CMs

spectrum_id:2967

2:

type:Specdb::CMs

spectrum_id:37274

3:

type:Specdb::CMs

spectrum_id:152885

4:

type:Specdb::NmrOneD

spectrum_id:4924

5:

type:Specdb::MsMs

spectrum_id:445885

6:

type:Specdb::MsMs

spectrum_id:445886

7:

type:Specdb::MsMs

spectrum_id:445887

8:

type:Specdb::MsMs

spectrum_id:445888

9:

type:Specdb::MsMs

spectrum_id:445889

10:

type:Specdb::MsMs

spectrum_id:447344

11:

type:Specdb::MsMs

spectrum_id:448029

12:

type:Specdb::MsMs

spectrum_id:448030

13:

type:Specdb::MsMs

spectrum_id:448139

14:

type:Specdb::MsMs

spectrum_id:344224

15:

type:Specdb::MsMs

spectrum_id:344225

16:

type:Specdb::MsMs

spectrum_id:344226

17:

type:Specdb::MsMs

spectrum_id:301675

18:

type:Specdb::MsMs

spectrum_id:301676

19:

type:Specdb::MsMs

spectrum_id:301677

20:

type:Specdb::MsMs

spectrum_id:2846036

21:

type:Specdb::MsMs

spectrum_id:2846037

22:

type:Specdb::MsMs

spectrum_id:2846038

光谱
生物学性质
1:

cellular:(1):Cytoplasm

cellular:(2):Endoplasmic reticulum

cellular:(3):Extracellular

cellular:(4):Mitochondria

cellular:(5):Peroxisome

2:

biospecimen:(1):Blood

biospecimen:(2):Breast Milk

biospecimen:(3):Cerebrospinal Fluid (CSF)

biospecimen:(4):Feces

biospecimen:(5):Saliva

biospecimen:(6):Urine

3:

tissue:(1):Adipose Tissue

tissue:(2):Bladder

tissue:(3):Brain

tissue:(4):Erythrocyte

tissue:(5):Fibroblasts

tissue:(6):Heart

tissue:(7):Intestine

tissue:(8):Kidney

tissue:(9):Liver

tissue:(10):Lung

tissue:(11):Neuron

tissue:(12):Placenta

tissue:(13):Platelet

tissue:(14):Prostate

tissue:(15):Semen

tissue:(16):Skeletal Muscle

tissue:(17):Testis

4:

[1]:

name:Adrenoleukodystrophy, X-linked

smpdb_id:SMP00516

kegg_map_id:

[2]:

name:Biosynthesis of unsaturated fatty acids

smpdb_id:

kegg_map_id:map01040

[3]:

name:Carnitine palmitoyl transferase deficiency (I)

smpdb_id:SMP00538

kegg_map_id:

[4]:

name:Carnitine palmitoyl transferase deficiency (II)

smpdb_id:SMP00541

kegg_map_id:

[5]:

name:Carnitine Synthesis

smpdb_id:SMP00465

kegg_map_id:

[6]:

name:Carnitine-acylcarnitine translocase deficiency

smpdb_id:SMP00517

kegg_map_id:

[7]:

name:Ethylmalonic Encephalopathy

smpdb_id:SMP00181

kegg_map_id:

[8]:

name:Fatty acid Metabolism

smpdb_id:SMP00051

kegg_map_id:map00071

[9]:

name:Glutaric Aciduria Type I

smpdb_id:SMP00185

kegg_map_id:

[10]:

name:Long chain acyl-CoA dehydrogenase deficiency (LCAD)

smpdb_id:SMP00539

kegg_map_id:

[11]:

name:Medium chain acyl-coa dehydrogenase deficiency (MCAD)

smpdb_id:SMP00542

kegg_map_id:

[12]:

name:Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids

smpdb_id:SMP00482

kegg_map_id:

[13]:

name:Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids

smpdb_id:SMP00480

kegg_map_id:

[14]:

name:Oxidation of Branched Chain Fatty Acids

smpdb_id:SMP00030

kegg_map_id:

[15]:

name:Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency)

smpdb_id:SMP00235

kegg_map_id:

[16]:

name:Trifunctional protein deficiency

smpdb_id:SMP00545

kegg_map_id:

[17]:

name:Very-long-chain acyl coa dehydrogenase deficiency (VLCAD)

smpdb_id:SMP00540

kegg_map_id:

正常浓度
1:

biospecimen:Blood

concentration_value:29.74 +/- 7.55

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

2:

biospecimen:Blood

concentration_value:25-88

concentration_units:uM

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

3:

biospecimen:Blood

concentration_value:28.500-109

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

4:

biospecimen:Blood

concentration_value:25.4-54.1

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

5:

biospecimen:Blood

concentration_value:40-60

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

6:

biospecimen:Blood

concentration_value:26-66

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

7:

biospecimen:Blood

concentration_value:28-47

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

8:

biospecimen:Blood

concentration_value:26-66

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

9:

biospecimen:Blood

concentration_value:37-58

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

10:

biospecimen:Blood

concentration_value:50 +/- 30

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

11:

biospecimen:Blood

concentration_value:>25

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

12:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

13:

biospecimen:Blood

concentration_value:26-70

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

14:

biospecimen:Blood

concentration_value:33.6 +/- 6.2

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

15:

biospecimen:Blood

concentration_value:38.2 +/- 5.4

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

16:

biospecimen:Blood

concentration_value:15.5 (13.2-17.7)

concentration_units:uM

subject_age:Newborn (0-30 days old)

subject_sex:

subject_condition:Normal

17:

biospecimen:Blood

concentration_value:16.28 +/- 8.41

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

18:

biospecimen:Blood

concentration_value:14.60 +/- 5.43

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Both

subject_condition:Normal

19:

biospecimen:Blood

concentration_value:43.0 (26.0-79.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

20:

biospecimen:Blood

concentration_value:41.2 (35.5-47.5)

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:

subject_condition:Normal

21:

biospecimen:Blood

concentration_value:43.57(9.97)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

22:

biospecimen:Blood

concentration_value:39.33(13.25)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

23:

biospecimen:Blood

concentration_value:40.430 +/- 3.650

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

24:

biospecimen:Blood

concentration_value:19.0-65.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

25:

biospecimen:Blood

concentration_value:35-75

concentration_units:uM

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

26:

biospecimen:Blood

concentration_value:35.3 +/- 7.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

27:

biospecimen:Blood

concentration_value:45.7 +/- 11.6

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

28:

biospecimen:Breast Milk

concentration_value:26.2 +/- 10.1

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

29:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:1.900 +/- 0.474

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

30:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:4.0 (2.0 - 9.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

31:

biospecimen:Feces

concentration_value:19.87 +/- 11.39

concentration_units:nmol/g wet feces

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

32:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

33:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

34:

biospecimen:Feces

concentration_value:8.13 +/- 3.11

concentration_units:nmol/g wet feces

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

35:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

36:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

37:

biospecimen:Saliva

concentration_value:5.24 +/- 1.035

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

38:

biospecimen:Saliva

concentration_value:0.924 +/- 0.960

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

39:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

40:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

41:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

42:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

43:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

44:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

45:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

46:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

47:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

48:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

49:

biospecimen:Saliva

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

50:

biospecimen:Saliva

concentration_value:0.656 +/- 0.734

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

51:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

52:

biospecimen:Urine

concentration_value:6926.20 +/- 754.66

concentration_units:umol/mmol creatinine

subject_age:Adolescent (13-18 years old)

subject_sex:Both

subject_condition:Normal

53:

biospecimen:Urine

concentration_value:10.733 +/- 1.733

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

54:

biospecimen:Urine

concentration_value:3.800-5.133

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

55:

biospecimen:Urine

concentration_value:0.317-40.928

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

56:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

57:

biospecimen:Urine

concentration_value:4.5 (0.62-15.2)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

58:

biospecimen:Urine

concentration_value:5.0 (0.7-16.4)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

59:

biospecimen:Urine

concentration_value:3.17

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

60:

biospecimen:Urine

concentration_value:5.7 +/- 4.8

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

61:

biospecimen:Urine

concentration_value:18.159 +/- 16.442

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

62:

biospecimen:Urine

concentration_value:0.79-5.66

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

63:

biospecimen:Urine

concentration_value:1.63(0.83-4.31)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Female

subject_condition:Normal

64:

biospecimen:Urine

concentration_value:1.80(0.78-5.85)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Male

subject_condition:Normal

65:

biospecimen:Urine

concentration_value:<100

concentration_units:umol/mmol creatinine

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

66:

biospecimen:Urine

concentration_value:23.5 +/- 4.0

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

67:

biospecimen:Urine

concentration_value:18.0 +/- 1.2

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

68:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

69:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

异常浓度
1:

biospecimen:Blood

concentration_value:22000-50000

concentration_units:uM

patient_age:Not Specified

patient_sex:Not Specified

patient_information:3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency

2:

biospecimen:Blood

concentration_value:41.7 +/- 23.9

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart Transplant

3:

biospecimen:Blood

concentration_value:17

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency

4:

biospecimen:Blood

concentration_value:47-68

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Female

patient_information:3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)

5:

biospecimen:Blood

concentration_value:20.6-33.8

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Myopathic carnitine deficiency

6:

biospecimen:Blood

concentration_value:0-2.2

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Female

patient_information:Long-chain Fatty Acids, Defect in Transport of

7:

biospecimen:Blood

concentration_value:12

concentration_units:uM

patient_age:Newborn (0-30 days old)

patient_sex:Male

patient_information:Carnitine palmitoyltransferase deficiency I

8:

biospecimen:Blood

concentration_value:0

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Female

patient_information:Carnitine transporter defect; primary systemic carnitine deficiency

9:

biospecimen:Blood

concentration_value:15

concentration_units:uM

patient_age:Newborn (0-30 days old)

patient_sex:Male

patient_information:Carnitine palmitoyltransferase deficiency I

10:

biospecimen:Blood

concentration_value:37-48

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Not Specified

patient_information:Oculocerebrorenal Syndrome of Lowe

11:

biospecimen:Blood

concentration_value:26.04 +/- 10.5

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy with fetuses with trisomy 18

12:

biospecimen:Blood

concentration_value:23-72

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Methylmalonic aciduria mitochondrial encephelopathy Leigh-like

13:

biospecimen:Blood

concentration_value:15-34

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Not Specified

patient_information:Oculocerebrorenal Syndrome of Lowe

14:

biospecimen:Blood

concentration_value:24.3 +/- 11.7

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

15:

biospecimen:Blood

concentration_value:9-33

concentration_units:uM

patient_age:Adolescent (13-18 years old)

patient_sex:Not Specified

patient_information:Oculocerebrorenal Syndrome of Lowe

16:

biospecimen:Blood

concentration_value:19-31

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Not Specified

patient_information:Oculocerebrorenal Syndrome of Lowe

17:

biospecimen:Blood

concentration_value:30.2 (6.3)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Early preeclampsia

18:

biospecimen:Blood

concentration_value:10-21

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:

patient_information:Mitochondrial trifunctional protein deficiency

19:

biospecimen:Blood

concentration_value:31.8 (14.1)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

20:

biospecimen:Blood

concentration_value:0.7

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Female

patient_information:Carnitine transporter defect; primary systemic carnitine deficiency

21:

biospecimen:Blood

concentration_value:4.2

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Male

patient_information:Carnitine transporter defect; primary systemic carnitine deficiency

22:

biospecimen:Blood

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

23:

biospecimen:Blood

concentration_value:43-51

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)

24:

biospecimen:Blood

concentration_value:16.798 (14.323)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy with fetus having congenital heart defect

25:

biospecimen:Blood

concentration_value:15.08 +/- 5.71

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Acetaminophen overdose

26:

biospecimen:Blood

concentration_value:21.08 (4.88)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy with fetus having congenital heart defect

27:

biospecimen:Blood

concentration_value:19.98 (4.82)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

28:

biospecimen:Blood

concentration_value:46.8 (24.8)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Late-onset preeclampsia

29:

biospecimen:Blood

concentration_value:18-22

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:2,4-dienoyl-CoA reductase deficiency

30:

biospecimen:Blood

concentration_value:27.8 (20.0)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

31:

biospecimen:Blood

concentration_value:50.65(14.14)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart failure with preserved ejection fraction

32:

biospecimen:Blood

concentration_value:47.22(15.19)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart failure with preserved ejection fraction

33:

biospecimen:Blood

concentration_value:54.38(13.17)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart failure with reduced ejection fraction

34:

biospecimen:Blood

concentration_value:50.38(8.11)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart failure with reduced ejection fraction

35:

biospecimen:Blood

concentration_value:20.540 +/- 1.850

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Not Specified

patient_information:3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency

36:

biospecimen:Blood

concentration_value:23-50

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:L-2-hydroxyglutaric aciduria

37:

biospecimen:Blood

concentration_value:34.0 +/- 8.8

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Obesity

38:

biospecimen:Blood

concentration_value:36.2 +/- 8.1

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:Obesity

39:

biospecimen:Blood

concentration_value:25.5 (9.7)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Down syndrome pregnancy

40:

biospecimen:Blood

concentration_value:19.5 (8.3)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Pregnancy

41:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

42:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

43:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal Cancer

44:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Oral cancer

45:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Breast cancer

46:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Not Specified

patient_information:Periodontal diseases

47:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Not Specified

patient_information:Pancreatic cancer

48:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Attachment loss 

49:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Missing teeth

50:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Periodontal Probing Depth

51:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Tooth Decay

52:

biospecimen:Urine

concentration_value:13.3 (11.6-15.1)

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Diabetes

53:

biospecimen:Urine

concentration_value:41.3 (38.3-44.2)

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Diabetes

54:

biospecimen:Urine

concentration_value:11183.81 +/- 6864.25

concentration_units:umol/mmol creatinine

patient_age:Adolescent (13-18 years old)

patient_sex:Both

patient_information:Obese

55:

biospecimen:Urine

concentration_value:1.517

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:

patient_information:Carnitine transporter defect; primary systemic carnitine deficiency

56:

biospecimen:Urine

concentration_value:9.395-121.200

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Myopathic carnitine deficiency

57:

biospecimen:Urine

concentration_value:7.21

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Not Specified

patient_information:Propionic acidemia

58:

biospecimen:Urine

concentration_value:7.0 (0.0-30.0)

concentration_units:umol/mmol creatinine

patient_age:Not Specified

patient_sex:Both

patient_information:Lung cancer

59:

biospecimen:Urine

concentration_value:17.565 +/- 24.221

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Eosinophilic esophagitis

60:

biospecimen:Urine

concentration_value:31-Jul

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Both

patient_information:L-2-hydroxyglutaric aciduria

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pubmed_id:19010317

18:

name:Periodontal disease

omim_id:170650

[1]:

reference_text:Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10.

pubmed_id:20300169

19:

name:Pancreatic cancer

omim_id:260350

[1]:

reference_text:Loser C, Folsch UR, Paprotny C, Creutzfeldt W: Polyamine concentrations in pancreatic tissue, serum, and urine of patients with pancreatic cancer. Pancreas. 1990 Mar;5(2):119-27.

pubmed_id:2315288

[2]:

reference_text:Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10.

pubmed_id:20300169

[3]:

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pubmed_id:25429707

20:

name:Attachment loss

omim_id:

[1]:

reference_text:Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26.

pubmed_id:31026179

21:

name:Missing teeth

omim_id:

[1]:

reference_text:Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26.

pubmed_id:31026179

22:

name:Periodontal Probing Depth

omim_id:

[1]:

reference_text:Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26.

pubmed_id:31026179

23:

name:Tooth Decay

omim_id:

[1]:

reference_text:Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26.

pubmed_id:31026179

24:

name:Diabetes mellitus type 2

omim_id:125853

[1]:

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pubmed_id:15899597

[2]:

reference_text:Costa A, Iguala I, Bedini J, Quinto L, Conget I: Uric acid concentration in subjects at risk of type 2 diabetes mellitus: relationship to components of the metabolic syndrome. Metabolism. 2002 Mar;51(3):372-5.

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pubmed_id:

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reference_text:Rosa Va ́zquez-Fresno et al. An NMR metabolomics approach reveals a combined-biomarkers model in a wine interventional trial with validation in free-living individuals of the PREDIMED study. Metabolomics (2015) 11:797- 806: https://link.springer.com/content/pdf/10.1007/s11306-014-0735-x.pdf

25:

name:Lung Cancer

omim_id:211980

[1]:

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pubmed_id:22157537

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pubmed_id:18953024

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pubmed_id:25961003

26:

name:Eosinophilic esophagitis

omim_id:610247

[1]:

reference_text:Mordechai, Hien, and David S. Wishart

pubmed_id:

27:

name:Propionic acidemia

omim_id:606054

[1]:

reference_text:Scholl-Burgi S, Haberlandt E, Gotwald T, Albrecht U, Baumgartner Sigl S, Rauchenzauner M, Rostasy K, Karall D: Stroke-like episodes in propionic acidemia caused by central focal metabolic decompensation. Neuropediatrics. 2009 Apr;40(2):76-81. doi: 10.1055/s-0029-1231065. Epub 2009 Oct 6.

pubmed_id:19809936

[2]:

reference_text:Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c.

pubmed_id:19551947

[3]:

reference_text:Thompson GN, Chalmers RA, Walter JH, Bresson JL, Lyonnet SL, Reed PJ, Saudubray JM, Leonard JV, Halliday D: The use of metronidazole in management of methylmalonic and propionic acidaemias. Eur J Pediatr. 1990 Aug;149(11):792-6.

pubmed_id:2226555

[4]:

reference_text:Riemersma M, Hazebroek MR, Helderman-van den Enden ATJM, Salomons GS, Ferdinandusse S, Brouwers MCGJ, van der Ploeg L, Heymans S, Glatz JFC, van den Wijngaard A, Krapels IPC, Bierau J, Brunner HG: Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017 Nov;25(11):1195-1201. doi: 10.1038/ejhg.2017.127. Epub 2017 Aug 30.

pubmed_id:28853722

[5]:

reference_text:MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

pubmed_id:

28:

name:Obesity

omim_id:601665

[1]:

reference_text:Tuma P, Samcova E, Balinova P: Determination of 3-methylhistidine and 1-methylhistidine in untreated urine samples by capillary electrophoresis. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Jul 5;821(1):53-9.

pubmed_id:15899597

[2]:

reference_text:Vice E, Privette JD, Hickner RC, Barakat HA: Ketone body metabolism in lean and obese women. Metabolism. 2005 Nov;54(11):1542-5.

pubmed_id:16253646

[3]:

reference_text:Brind J, Strain G, Miller L, Zumoff B, Vogelman J, Orentreich N: Obese men have elevated plasma levels of estrone sulfate. Int J Obes. 1990 Jun;14(6):483-6.

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reference_text:Serlie MJ, Meijer AJ, Groener JE, Duran M, Endert E, Fliers E, Aerts JM, Sauerwein HP: Short-term manipulation of plasma free fatty acids does not change skeletal muscle concentrations of ceramide and glucosylceramide in lean and overweight subjects. J Clin Endocrinol Metab. 2007 Apr;92(4):1524-9. Epub 2007 Jan 30.

pubmed_id:17264178

[5]:

reference_text:Driskell JA, Chrisley BM, Reynolds LK, Moak SW: Plasma B6 vitamer and plasma and urinary 4-pyridoxic acid concentrations of middle-aged obese black women. J Chromatogr. 1991 Aug 23;568(2):333-40.

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reference_text:Haro C, Montes-Borrego M, Rangel-Zuniga OA, Alcala-Diaz JF, Gomez-Delgado F, Perez-Martinez P, Delgado-Lista J, Quintana-Navarro GM, Tinahones FJ, Landa BB, Lopez-Miranda J, Camargo A, Perez-Jimenez F: Two Healthy Diets Modulate Gut Microbial Community Improving Insulin Sensitivity in a Human Obese Population. J Clin Endocrinol Metab. 2016 Jan;101(1):233-42. doi: 10.1210/jc.2015-3351. Epub 2015 Oct 27.

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reference_text:Vioque J, Weinbrenner T, Asensio L, Castello A, Young IS, Fletcher A: Plasma concentrations of carotenoids and vitamin C are better correlated with dietary intake in normal weight than overweight and obese elderly subjects. Br J Nutr. 2007 May;97(5):977-86.

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[8]:

reference_text:Burrows TL, Warren JM, Colyvas K, Garg ML, Collins CE: Validation of overweight children's fruit and vegetable intake using plasma carotenoids. Obesity (Silver Spring). 2009 Jan;17(1):162-8. doi: 10.1038/oby.2008.495. Epub 2008 Nov 6.

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[9]:

reference_text:Reinehr T, Wolters B, Knop C, Lass N, Hellmuth C, Harder U, Peissner W, Wahl S, Grallert H, Adamski J, Illig T, Prehn C, Yu Z, Wang-Sattler R, Koletzko B: Changes in the serum metabolite profile in obese children with weight loss. Eur J Nutr. 2015 Mar;54(2):173-81. doi: 10.1007/s00394-014-0698-8. Epub 2014 Apr 17.

pubmed_id:24740590

[10]:

reference_text:Wahl S, Yu Z, Kleber M, Singmann P, Holzapfel C, He Y, Mittelstrass K, Polonikov A, Prehn C, Romisch-Margl W, Adamski J, Suhre K, Grallert H, Illig T, Wang-Sattler R, Reinehr T: Childhood obesity is associated with changes in the serum metabolite profile. Obes Facts. 2012;5(5):660-70. doi: 10.1159/000343204. Epub 2012 Oct 4.

pubmed_id:23108202

[11]:

reference_text:Cho K, Moon JS, Kang JH, Jang HB, Lee HJ, Park SI, Yu KS, Cho JY: Combined untargeted and targeted metabolomic profiling reveals urinary biomarkers for discriminating obese from normal-weight adolescents. Pediatr Obes. 2017 Apr;12(2):93-101. doi: 10.1111/ijpo.12114. Epub 2016 Feb 22.

pubmed_id:26910390

[12]:

reference_text:Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children

pubmed_id:

KEGG数据库编号
C00318
DrugBank数据库编号
DB00583
foodb数据库编号
FDB000571
ChemSpider数据库编号
2006614
公共化学化合物编号
10917
PDB数据库编号
生物利益的化学实体数据库编号
16347
knapsack数据库编号
维基百科编号
Carnitine
代谢途径的数据库编号
CARNITINE
苯酚资源管理器化合物数据库编号
比格数据库编号
34600
梅林编号
52
虚拟机身份证编号
CRN
fbonto数据库编号
综合参考
Bols, Mikael; Lundt, Inge; Pedersen, Christian. Simple synthesis of (R)-carnitine from D-galactono-1,4-lactone. Tetrahedron (1992), 48(2), 319-24.
一般参考
1:

protein_accession:HMDBP00028

name:Carnitine O-acetyltransferase

uniprot_id:P43155

gene_name:CRAT

protein_type:Enzyme

2:

protein_accession:HMDBP00181

name:Xanthine dehydrogenase/oxidase

uniprot_id:P47989

gene_name:XDH

protein_type:Unknown

3:

protein_accession:HMDBP00282

name:Myeloperoxidase

uniprot_id:P05164

gene_name:MPO

protein_type:Unknown

4:

protein_accession:HMDBP00307

name:Peroxisomal carnitine O-octanoyltransferase

uniprot_id:Q9UKG9

gene_name:CROT

protein_type:Enzyme

5:

protein_accession:HMDBP00308

name:Carnitine O-palmitoyltransferase 1, muscle isoform

uniprot_id:Q92523

gene_name:CPT1B

protein_type:Enzyme

6:

protein_accession:HMDBP00309

name:Carnitine O-palmitoyltransferase 1, liver isoform

uniprot_id:P50416

gene_name:CPT1A

protein_type:Enzyme

7:

protein_accession:HMDBP00311

name:Carnitine O-palmitoyltransferase 2, mitochondrial

uniprot_id:P23786

gene_name:CPT2

protein_type:Enzyme

8:

protein_accession:HMDBP00312

name:Carnitine O-palmitoyltransferase 1, brain isoform

uniprot_id:Q8TCG5

gene_name:CPT1C

protein_type:Enzyme

9:

protein_accession:HMDBP00382

name:Gamma-butyrobetaine dioxygenase

uniprot_id:O75936

gene_name:BBOX1

protein_type:Unknown

10:

protein_accession:HMDBP00454

name:Solute carrier organic anion transporter family member 1B1

uniprot_id:Q9Y6L6

gene_name:SLCO1B1

protein_type:Transporter

11:

protein_accession:HMDBP00497

name:Liver carboxylesterase 1

uniprot_id:P23141

gene_name:CES1

protein_type:Enzyme

12:

protein_accession:HMDBP01612

name:Solute carrier family 22 member 5

uniprot_id:O76082

gene_name:SLC22A5

protein_type:Transporter

13:

protein_accession:HMDBP02439

name:Solute carrier family 22 member 4

uniprot_id:Q9H015

gene_name:SLC22A4

protein_type:Transporter

14:

protein_accession:HMDBP02794

name:Mitochondrial carnitine/acylcarnitine carrier protein

uniprot_id:O43772

gene_name:SLC25A20

protein_type:Enzyme

15:

protein_accession:HMDBP02811

name:Solute carrier family 22 member 16

uniprot_id:Q86VW1

gene_name:SLC22A16

protein_type:Transporter

16:

protein_accession:HMDBP10761

name:Mitochondrial carnitine/acylcarnitine carrier protein CACL

uniprot_id:Q8N8R3

gene_name:SLC25A29

protein_type:Unknown

蛋白质结合
1:

reference_text:Tamai I, China K, Sai Y, Kobayashi D, Nezu J, Kawahara E, Tsuji A: Na(+)-coupled transport of L-carnitine via high-affinity carnitine transporter OCTN2 and its subcellular localization in kidney. Biochim Biophys Acta. 2001 Jun 6;1512(2):273-84.

pubmed_id:11406104

2:

reference_text:Waldner R, Laschan C, Lohninger A, Gessner M, Tuchler H, Huemer M, Spiegel W, Karlic H: Effects of doxorubicin-containing chemotherapy and a combination with L-carnitine on oxidative metabolism in patients with non-Hodgkin lymphoma. J Cancer Res Clin Oncol. 2006 Feb;132(2):121-8. Epub 2005 Nov 8.

pubmed_id:16283381

3:

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