版本
5.0
创建日期
2005-11-16 15:48:42 UTC
更新日期
2020-11-09 23:13:04 UTC
登录号
HMDB0000251
地位
quantified
二级访问
HMDB00251

名称
Taurine
描述
Taurine is a sulfur amino acid like methionine, cystine, cysteine, and homocysteine. It is a lesser-known amino acid because it is not incorporated into the structural building blocks of protein. Yet taurine is an essential amino acid in pre-term and newborn infants of humans and many other species. Adults can synthesize their own taurine, yet are probably dependent, in part, on dietary taurine. Taurine is abundant in the brain, heart, breast, gallbladder, and kidney and has important roles in health and disease in these organs. Taurine has many diverse biological functions including serving as a neurotransmitter in the brain, a stabilizer of cell membranes, and a facilitator in the transport of ions such as sodium, potassium, calcium, and magnesium. Taurine is highly concentrated in animal and fish protein, which are good sources of dietary taurine. It can be synthesized by the body from cysteine when vitamin B6 is present. Deficiency of taurine occurs in premature infants, neonates fed formula milk, and various disease states. Several inborn errors of taurine metabolism have been described. Perry syndrome is an unusual neuropsychiatric disorder inherited in an autosomal dominant fashion through three generations of a family. Symptoms began late in the fifth decade in 6 affected persons and death occurred after 4 to 6 years. The earliest and most prominent symptom was mental depression that was not responsive to antidepressant drugs or electroconvulsive therapy. Sleep disturbances, exhaustion, and marked weight loss were features. Parkinsonism developed later, and respiratory failure occurred terminally (OMIM: 168605). Hypertaurinuric cardiomyopathy describes congestive cardiomyopathy and markedly elevated urinary taurine levels (about 5 times normal). Other family members had late or holosystolic mitral valve prolapse and elevated urinary taurine values (about 2.5 times normal). In two with mitral valve prolapse, congestive cardiomyopathy eventually developed while the amounts of urinary taurine doubled (OMIM: 145350). Taurine, after GABA, is the second most important inhibitory neurotransmitter in the brain. Its inhibitory effect is one source of taurine's anticonvulsant and antianxiety properties. It also lowers glutamic acid in the brain, and preliminary clinical trials suggest taurine may be useful in some forms of epilepsy. Taurine in the brain is usually associated with zinc or manganese. The amino acids alanine and glutamic acid, as well as pantothenic acid, inhibit taurine metabolism while vitamins A and B6, zinc, and manganese help build taurine. Cysteine and B6 are the nutrients most directly involved in taurine synthesis. Taurine levels have been found to decrease significantly in many depressed patients. One reason that the findings are not entirely clear is that taurine is often elevated in the blood of epileptics who need it. It is often difficult to distinguish compensatory changes in human biochemistry from true metabolic or deficiency disease. Low levels of taurine are found in retinitis pigmentosa. Taurine deficiency in experimental animals produces degeneration of light-sensitive cells. Therapeutic applications of taurine to eye disease are likely to be forthcoming. Taurine has many important metabolic roles. Supplements can stimulate prolactin and insulin release. The parathyroid gland makes a peptide hormone called glutataurine (glutamic acid-taurine), which further demonstrates taurine's role in endocrinology. Taurine increases bilirubin and cholesterol excretion in bile, critical to normal gallbladder function. It seems to inhibit the effect of morphine and potentiates the effects of opiate antagonists. Low plasma taurine levels have been found in a variety of conditions, i.e. depression, hypertension, hypothyroidism, gout, institutionalized patients, infertility, obesity, kidney failure, and others (http://www.dcnutrition.com/AminoAcids/). Moreover, taurine is found to be associated with maple syrup urine disease, which is an inborn error of metabolism.
同义词
1:2-Aminoethanesulfonic acid

2:2-Aminoethyl sulfonate

3:Aminoethylsulfonic acid

4:beta-Aminoethylsulfonic acid

5:2-Aminoethanesulfonate

6:2-Aminoethanesulphonate

7:2-Aminoethanesulphonic acid

8:2-Aminoethyl sulfonic acid

9:2-Aminoethyl sulphonate

10:2-Aminoethyl sulphonic acid

11:Aminoethylsulfonate

12:Aminoethylsulphonate

13:Aminoethylsulphonic acid

14:b-Aminoethylsulfonate

15:b-Aminoethylsulfonic acid

16:b-Aminoethylsulphonate

17:b-Aminoethylsulphonic acid

18:beta-Aminoethylsulfonate

19:beta-Aminoethylsulphonate

20:beta-Aminoethylsulphonic acid

21:Β-aminoethylsulfonate

22:Β-aminoethylsulfonic acid

23:Β-aminoethylsulphonate

24:Β-aminoethylsulphonic acid

25:1-Aminoethane-2-sulfonate

26:1-Aminoethane-2-sulfonic acid

27:2-Aminoethylsulfonate

28:2-Aminoethylsulfonic acid

29:2-Sulfoethylamine

30:Taurine hydrochloride

31:Taurine zinc salt (2:1)

32:Taurine, monopotassium salt

33:Taufon

34:Tauphon

化学式
C2H7NO3S
平均分子量
125.147
等位分子量
125.014663785
化学名称
2-aminoethane-1-sulfonic acid
传统名称
taurine
CAS 登记号
107-35-7
代谢物结构字符串
NCCS(O)(=O)=O
inchi标识符
InChI=1S/C2H7NO3S/c3-1-2-7(4,5)6/h1-3H2,(H,4,5,6)
印记键
XOAAWQZATWQOTB-UHFFFAOYSA-N
分类学
description: belongs to the class of organic compounds known as organosulfonic acids. Organosulfonic acids are compounds containing the sulfonic acid group, which has the general structure RS(=O)2OH (R is not a hydrogen atom).

description:Organosulfonic acids

kingdom:Organic compounds

super_class:Organic acids and derivatives

class:Organic sulfonic acids and derivatives

sub_class:Organosulfonic acids and derivatives

molecular_framework:Aliphatic acyclic compounds

1:Alkanesulfonic acids

2:Hydrocarbon derivatives

3:Monoalkylamines

4:Organic oxides

5:Organopnictogen compounds

6:Sulfonyls

7:Aliphatic acyclic compound

8:Alkanesulfonic acid

9:Amine

10:Hydrocarbon derivative

11:Organic nitrogen compound

12:Organic oxide

13:Organic oxygen compound

14:Organonitrogen compound

15:Organopnictogen compound

16:Organosulfonic acid

17:Organosulfur compound

18:Primary aliphatic amine

19:Primary amine

20:Sulfonyl

21:amino sulfonic acid

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: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:Epilepsy

definition:A brain disease that is characterized by the occurrance of at least two unprovoked seizures resulting from a persistent epileptogenic abnormality of the brain that is able to spontaneously generate paroxysmal activity and typically manifested by sudden brief episodes of altered or diminished consciousness, involuntary movements, or convulsions. (do).

parent_id:7695

***level:4

type:child

synonym:Epileptic spasms

term:Schizophrenia

definition:A psychotic disorder that is characterized by a disintegration of thought processes and of emotional responsiveness. (do).

parent_id:7695

***level:4

type:child

term:Cardiac disorders

definition:Any disorder that affects the heart.

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: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: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: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: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:Pancreas

definition:A digestive organ in the abdomen that has both endocrine and exocrine functions. (nci).

parent_id:7727

***level:4

type:child

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: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:Epidermis

definition:The epithelial portion of the skin (cutis). it consists of the following layers: stratum corneum (horny layer), stratum granulosum (granular layer), stratum spinosum (prickle cell layer), and stratum basale (basal cell layer). (nci).

parent_id:7729

***level:4

type:child

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:Nervous tissue

definition:The tissue that generates and conducts electrical signals in the body. it contains the neurons. (nci).

parent_id:7729

***level:4

type:child

synonym:Neural tissue

synonym:Nerve tissue

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:Biofluid and excreta

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

parent_id:7725

**level:3

type:parent

term:Bile

definition:Fluid composed of waste products, bile acids, salts, cholesterol, and electrolytes. it is secreted by the liver parenchyma and stored in the gallbladder. (nci).

parent_id:7731

***level:4

type:child

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: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:Subcellular

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

parent_id:7725

**level:3

type:parent

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: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: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:Food and nutrition

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

parent_id:7678

**level:3

type:child

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:Osmolyte

definition:A chemical substance that affects osmosis. osmolytes are soluble in the cell or in the fluid between cells, and are utilized by cells during water stress to maintain the cell volume.

parent_id:7672

**level:3

type:child

synonym:Osmolosis agent

term:Essential amino acid

definition:

parent_id:7672

**level:3

type:child

term:Antioxidant

definition:a chemical substance that is used to maintain the quality, integrity, and safety of finished products by inhibiting the oxidative degradation of the ingredients in the formulation. Also referred to as a preservative

parent_id:7672

**level:3

type:child

synonym:Anti-oxidant

状态
Solid
实验性质
1:

kind:water_solubility

value:80.7 mg/mL

source:

2:

kind:melting_point

value:300 °C

source:

预测性质
1:

kind:logp

value:-2.19

source:ALOGPS

2:

kind:logs

value:-0.08

source:ALOGPS

3:

kind:solubility

value:105 g/L

source:ALOGPS

4:

kind:logp

value:-2.6

source:ChemAxon

5:

kind:pka_strongest_acidic

value:-1.5

source:ChemAxon

6:

kind:pka_strongest_basic

value:9.34

source:ChemAxon

7:

kind:iupac

value:2-aminoethane-1-sulfonic acid

source:ChemAxon

8:

kind:average_mass

value:125.147

source:ChemAxon

9:

kind:mono_mass

value:125.014663785

source:ChemAxon

10:

kind:smiles

value:NCCS(O)(=O)=O

source:ChemAxon

11:

kind:formula

value:C2H7NO3S

source:ChemAxon

12:

kind:inchi

value:InChI=1S/C2H7NO3S/c3-1-2-7(4,5)6/h1-3H2,(H,4,5,6)

source:ChemAxon

13:

kind:inchikey

value:XOAAWQZATWQOTB-UHFFFAOYSA-N

source:ChemAxon

14:

kind:polar_surface_area

value:80.39

source:ChemAxon

15:

kind:refractivity

value:24.61

source:ChemAxon

16:

kind:polarizability

value:10.82

source:ChemAxon

17:

kind:rotatable_bond_count

value:2

source:ChemAxon

18:

kind:acceptor_count

value:4

source:ChemAxon

19:

kind:donor_count

value:2

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::EiMs

spectrum_id:1313

2:

type:Specdb::NmrOneD

spectrum_id:1218

3:

type:Specdb::NmrOneD

spectrum_id:1277

4:

type:Specdb::NmrOneD

spectrum_id:2107

5:

type:Specdb::NmrOneD

spectrum_id:4964

6:

type:Specdb::NmrOneD

spectrum_id:6272

7:

type:Specdb::NmrOneD

spectrum_id:6273

8:

type:Specdb::NmrOneD

spectrum_id:6274

9:

type:Specdb::NmrOneD

spectrum_id:6275

10:

type:Specdb::NmrOneD

spectrum_id:6276

11:

type:Specdb::NmrOneD

spectrum_id:6277

12:

type:Specdb::NmrOneD

spectrum_id:6278

13:

type:Specdb::NmrOneD

spectrum_id:6279

14:

type:Specdb::NmrOneD

spectrum_id:6280

15:

type:Specdb::NmrOneD

spectrum_id:6281

16:

type:Specdb::NmrOneD

spectrum_id:6282

17:

type:Specdb::NmrOneD

spectrum_id:6283

18:

type:Specdb::NmrOneD

spectrum_id:6284

19:

type:Specdb::NmrOneD

spectrum_id:6285

20:

type:Specdb::NmrOneD

spectrum_id:6286

21:

type:Specdb::NmrOneD

spectrum_id:6287

22:

type:Specdb::NmrOneD

spectrum_id:6288

23:

type:Specdb::NmrOneD

spectrum_id:6289

24:

type:Specdb::NmrOneD

spectrum_id:6290

25:

type:Specdb::NmrOneD

spectrum_id:6291

26:

type:Specdb::NmrOneD

spectrum_id:166456

27:

type:Specdb::MsMs

spectrum_id:430

28:

type:Specdb::MsMs

spectrum_id:431

29:

type:Specdb::MsMs

spectrum_id:432

30:

type:Specdb::MsMs

spectrum_id:3742

31:

type:Specdb::MsMs

spectrum_id:3743

32:

type:Specdb::MsMs

spectrum_id:3744

33:

type:Specdb::MsMs

spectrum_id:3745

34:

type:Specdb::MsMs

spectrum_id:3746

35:

type:Specdb::MsMs

spectrum_id:3747

36:

type:Specdb::MsMs

spectrum_id:3748

37:

type:Specdb::MsMs

spectrum_id:3749

38:

type:Specdb::MsMs

spectrum_id:3750

39:

type:Specdb::MsMs

spectrum_id:3751

40:

type:Specdb::MsMs

spectrum_id:3752

41:

type:Specdb::MsMs

spectrum_id:3753

42:

type:Specdb::MsMs

spectrum_id:3754

43:

type:Specdb::MsMs

spectrum_id:3755

44:

type:Specdb::MsMs

spectrum_id:3756

45:

type:Specdb::MsMs

spectrum_id:3757

46:

type:Specdb::MsMs

spectrum_id:3758

47:

type:Specdb::MsMs

spectrum_id:3759

48:

type:Specdb::MsMs

spectrum_id:3760

49:

type:Specdb::MsMs

spectrum_id:3761

50:

type:Specdb::MsMs

spectrum_id:3762

51:

type:Specdb::MsMs

spectrum_id:3763

52:

type:Specdb::NmrTwoD

spectrum_id:1007

53:

type:Specdb::NmrTwoD

spectrum_id:1238

54:

type:Specdb::CMs

spectrum_id:487

55:

type:Specdb::CMs

spectrum_id:488

56:

type:Specdb::CMs

spectrum_id:489

57:

type:Specdb::CMs

spectrum_id:2519

58:

type:Specdb::CMs

spectrum_id:30234

59:

type:Specdb::CMs

spectrum_id:30698

60:

type:Specdb::CMs

spectrum_id:30763

61:

type:Specdb::CMs

spectrum_id:32381

62:

type:Specdb::CMs

spectrum_id:32383

63:

type:Specdb::CMs

spectrum_id:166328

光谱
生物学性质
1:

cellular:(1):Extracellular

cellular:(2):Peroxisome

2:

biospecimen:(1):Bile

biospecimen:(2):Blood

biospecimen:(3):Breast Milk

biospecimen:(4):Cerebrospinal Fluid (CSF)

biospecimen:(5):Feces

biospecimen:(6):Saliva

biospecimen:(7):Urine

3:

tissue:(1):Brain

tissue:(2):Epidermis

tissue:(3):Erythrocyte

tissue:(4):Fibroblasts

tissue:(5):Intestine

tissue:(6):Kidney

tissue:(7):Leukocyte

tissue:(8):Liver

tissue:(9):Neuron

tissue:(10):Pancreas

tissue:(11):Placenta

tissue:(12):Platelet

tissue:(13):Prostate

tissue:(14):Retina

tissue:(15):Skeletal Muscle

4:

[1]:

name:27-Hydroxylase Deficiency

smpdb_id:SMP00720

kegg_map_id:

[2]:

name:Cerebrotendinous Xanthomatosis (CTX)

smpdb_id:SMP00315

kegg_map_id:

[3]:

name:Congenital Bile Acid Synthesis Defect Type II

smpdb_id:SMP00314

kegg_map_id:

[4]:

name:Congenital Bile Acid Synthesis Defect Type III

smpdb_id:SMP00318

kegg_map_id:

[5]:

name:Familial Hypercholanemia (FHCA)

smpdb_id:SMP00317

kegg_map_id:

[6]:

name:Primary bile acid biosynthesis

smpdb_id:

kegg_map_id:map00120

[7]:

name:Taurine and Hypotaurine Metabolism

smpdb_id:SMP00021

kegg_map_id:map00430

[8]:

name:Zellweger Syndrome

smpdb_id:SMP00316

kegg_map_id:

正常浓度
1:

biospecimen:Bile

concentration_value:>10

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

2:

biospecimen:Blood

concentration_value:0-360

concentration_units:uM

subject_age:Infant (0-1 year old)

subject_sex:Both

subject_condition:Normal

3:

biospecimen:Blood

concentration_value:55.0 (42.0-69.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

4:

biospecimen:Blood

concentration_value:61 +/- 17

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

5:

biospecimen:Blood

concentration_value:42 +/- 12

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

6:

biospecimen:Blood

concentration_value:104.0 +/- 62.0

concentration_units:uM

subject_age:Children (1-13 years old)

subject_sex:Male

subject_condition:Normal

7:

biospecimen:Blood

concentration_value:162.0 +/- 60.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

8:

biospecimen:Blood

concentration_value:141.0 +/- 57.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

9:

biospecimen:Blood

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

10:

biospecimen:Blood

concentration_value:140 +/- 40

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

11:

biospecimen:Blood

concentration_value:70.00-170.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

12:

biospecimen:Blood

concentration_value:93.0 +/- 35.7

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

13:

biospecimen:Blood

concentration_value:152.1(113.7-179.8)

concentration_units:uM

subject_age:Children (1-13 uears old)

subject_sex:Both

subject_condition:Normal

14:

biospecimen:Blood

concentration_value:91.0 +/- 3.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

15:

biospecimen:Blood

concentration_value:61.0 +/- 17.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

16:

biospecimen:Blood

concentration_value:42.0 +/- 12.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

17:

biospecimen:Blood

concentration_value:19-265

concentration_units:uM

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

18:

biospecimen:Blood

concentration_value:19-190

concentration_units:uM

subject_age:Infant (1 - 3 months old)

subject_sex:Both

subject_condition:Normal

19:

biospecimen:Blood

concentration_value:10-97

concentration_units:uM

subject_age:Children (3 months - 6 years old)

subject_sex:Both

subject_condition:Normal

20:

biospecimen:Blood

concentration_value:0-240

concentration_units:uM

subject_age:Children (6 - 18 years old)

subject_sex:Both

subject_condition:Normal

21:

biospecimen:Breast Milk

concentration_value:190 +/- 66.6

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

22:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:6.6 +/- 1.7

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

23:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:8.24 (5.48-11.0)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

24:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:13.4 +/- 2.7

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

25:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:8.4 +/- 2.0

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

26:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:5.5 +/- 1.5

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

27:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:0-16

concentration_units:uM

subject_age:Children (0 - 10 years old)

subject_sex:Both

subject_condition:Normal

28:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:0-14

concentration_units:uM

subject_age:Adolescent (>11 years old)

subject_sex:Both

subject_condition:Normal

29:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:5.87 +/- 0.63

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

30:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:5.3 +/- 2.2

concentration_units:uM

subject_age:Not Specified

subject_sex:Not Specified

subject_condition:Normal

31:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

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:Not Specified

subject_sex:Both

subject_condition:Normal

34:

biospecimen:Feces

concentration_value:

concentration_units:

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:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

37:

biospecimen:Feces

concentration_value:140 +/- 290

concentration_units:nmol/g wet feces

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

38:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Children (6 - 18 years old)

subject_sex:Both

subject_condition:Normal

39:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Children (6 - 18 years old)

subject_sex:Not Specified

subject_condition:Normal

40:

biospecimen:Feces

concentration_value:10 +/- 10

concentration_units:nmol/g wet feces

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

41:

biospecimen:Feces

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

42:

biospecimen:Saliva

concentration_value:40.1 +/- 24.5

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

43:

biospecimen:Saliva

concentration_value:105.56 +/- 56.53

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

44:

biospecimen:Saliva

concentration_value:57.8 +/- 24.3

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

subject_condition:Normal

45:

biospecimen:Saliva

concentration_value:52 (10-268)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

46:

biospecimen:Saliva

concentration_value:66 (1-171)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

47:

biospecimen:Saliva

concentration_value:45 (<1-184)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

48:

biospecimen:Saliva

concentration_value:25 (<1-69)

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Female

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:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

51:

biospecimen:Saliva

concentration_value:1-418

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:normal

52:

biospecimen:Saliva

concentration_value:1-56

concentration_units:uM

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:normal

53:

biospecimen:Urine

concentration_value:350.49 +/- 192.20

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

54:

biospecimen:Urine

concentration_value:463.55 +/- 214.81

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

55:

biospecimen:Urine

concentration_value:520.08 +/- 293.96

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

56:

biospecimen:Urine

concentration_value:54.7

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

57:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

58:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Male

subject_condition:Normal

59:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

60:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

61:

biospecimen:Urine

concentration_value:74.64-2841

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

62:

biospecimen:Urine

concentration_value:1114 +/- 761.3

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Female

subject_condition:Normal

63:

biospecimen:Urine

concentration_value:6.45-252.82

concentration_units:umol/mmol creatinine

subject_age:Adolescent (13-18 years old)

subject_sex:Not Specified

subject_condition:Normal

64:

biospecimen:Urine

concentration_value:1042 +/- 591.5

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Male

subject_condition:Normal

65:

biospecimen:Urine

concentration_value:

concentration_units:

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

66:

biospecimen:Urine

concentration_value:4.00-159.98

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

67:

biospecimen:Urine

concentration_value:2.71-173.18

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Not Specified

subject_condition:Normal

68:

biospecimen:Urine

concentration_value:4.18-938.73

concentration_units:umol/mmol creatinine

subject_age:Infant (0-1 year old)

subject_sex:Not Specified

subject_condition:Normal

69:

biospecimen:Urine

concentration_value:8.37 - 293.50

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

70:

biospecimen:Urine

concentration_value:5.65 - 124.48

concentration_units:umol/mmol creatinine

subject_age:Infant (1 - 6 months old)

subject_sex:Both

subject_condition:Normal

71:

biospecimen:Urine

concentration_value:9.72 - 158.62

concentration_units:umol/mmol creatinine

subject_age:Infant (6 months - <1 year old)

subject_sex:Both

subject_condition:Normal

72:

biospecimen:Urine

concentration_value:13.57 - 205.77

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 2 years old)

subject_sex:Both

subject_condition:Normal

73:

biospecimen:Urine

concentration_value:14.81 - 259.47

concentration_units:umol/mmol creatinine

subject_age:Children (2 - 4 years old)

subject_sex:Both

subject_condition:Normal

74:

biospecimen:Urine

concentration_value:20.01 - 298.59

concentration_units:umol/mmol creatinine

subject_age:Children (4 - 13 years old)

subject_sex:Both

subject_condition:Normal

75:

biospecimen:Urine

concentration_value:18.65 - 233.13

concentration_units:umol/mmol creatinine

subject_age:Adolescent (13 - 21 years old)

subject_sex:Both

subject_condition:Normal

76:

biospecimen:Urine

concentration_value:18.65 - 233.13

concentration_units:umol/mmol creatinine

subject_age:Adult (>21 years old)

subject_sex:Both

subject_condition:Normal

77:

biospecimen:Urine

concentration_value:90.239 +/- 61.212

concentration_units:umol/mmol creatinine

subject_age:Children (1 - 13 years old)

subject_sex:Not Specified

subject_condition:Normal

78:

biospecimen:Urine

concentration_value:57.42

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

79:

biospecimen:Urine

concentration_value:81 (13-251)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

80:

biospecimen:Urine

concentration_value:7.24-368.20

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

81:

biospecimen:Urine

concentration_value:1.6 +/- 1.6

concentration_units:umol/mmol creatinine

subject_age:Infant (1 - 6 months old)

subject_sex:Both

subject_condition:Normal

82:

biospecimen:Urine

concentration_value:2.5 +/-3.2

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

83:

biospecimen:Urine

concentration_value:3.2 +/- 3.1

concentration_units:umol/mmol creatinine

subject_age:Infant (6 months - <1 year old)

subject_sex:Both

subject_condition:Normal

84:

biospecimen:Urine

concentration_value:36.07 +/- 29.16

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

85:

biospecimen:Urine

concentration_value:60.45 (17.78-103.11)

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

86:

biospecimen:Urine

concentration_value:8.60-398.062

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

87:

biospecimen:Urine

concentration_value:63.2 (21.1-105.0)

concentration_units:umol/mmol creatinine

subject_age:Adult (>18 years old)

subject_sex:Both

subject_condition:Normal

88:

biospecimen:Urine

concentration_value:250-910

concentration_units:umol/mmol creatinine

subject_age:Newborn (0-30 days old)

subject_sex:Both

subject_condition:Normal

89:

biospecimen:Urine

concentration_value:5.66-232.0048

concentration_units:umol/mmol creatinine

subject_age:Children (1-13 years old)

subject_sex:Not Specified

subject_condition:Normal

异常浓度
1:

biospecimen:Blood

concentration_value:188

concentration_units:uM

patient_age:Infant (0-1 year old)

patient_sex:Male

patient_information:Sulfite oxidase deficiency

2:

biospecimen:Blood

concentration_value:117.0 +/- 7.0

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Heart failure

3:

biospecimen:Blood

concentration_value:67 +/- 20

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Schizophrenia

4:

biospecimen:Blood

concentration_value:68 +/- 14

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Schizophrenia

5:

biospecimen:Blood

concentration_value:182.7(150.9-226.9)

concentration_units:uM

patient_age:Children (1-13 uears old)

patient_sex:Both

patient_information:Environmental enteric dysfunction

6:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:5.80 (5.30-6.30)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Epilepsy

7:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:6.49 (4.60-8.38)

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Parkinson's Disease

8:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:8.0 +/- 4.3

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Not Specified

patient_information:Leukemia

9:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:10.4 +/- 11.1

concentration_units:uM

patient_age:Children (1-13 years old)

patient_sex:Not Specified

patient_information:Leukemia

10:

biospecimen:Cerebrospinal Fluid (CSF)

concentration_value:5.0 +/- 0.86

concentration_units:uM

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

11:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Male

patient_information:Gout

12:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

13:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal Cancer

14:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Ulcerative colitis

15:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:ankylosing spondylitis

16:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:rheumatoid arthritis

17:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Colorectal cancer

18:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Irritable bowel syndrome

19:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Ulcerative colitis

20:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Children (6 - 18 years old)

patient_sex:Both

patient_information:Crohns disease

21:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Children (6 - 18 years old)

patient_sex:Both

patient_information:Ulcerative colitis

22:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Children (6 - 18 years old)

patient_sex:Not Specified

patient_information:Crohns disease

23:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Children (6 - 18 years old)

patient_sex:Not Specified

patient_information:Ulcerative colitis

24:

biospecimen:Feces

concentration_value:

concentration_units:

patient_age:Children (6 - 18 years old)

patient_sex:Not Specified

patient_information:Unclassified IBD

25:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Oral cancer

26:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Breast cancer

27:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Not Specified

patient_information:Pancreatic cancer

28:

biospecimen:Saliva

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Not Specified

patient_information:Periodontal diseases

29:

biospecimen:Urine

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Female

patient_information:Epithelial ovarian cancer

30:

biospecimen:Urine

concentration_value:

concentration_units:

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Schizophrenia

31:

biospecimen:Urine

concentration_value:58.393 +/- 30.929

concentration_units:umol/mmol creatinine

patient_age:Children (1 - 13 years old)

patient_sex:Not Specified

patient_information:Eosinophilic esophagitis

32:

biospecimen:Urine

concentration_value:0.0027 - 0.075

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:ADPKD

33:

biospecimen:Urine

concentration_value:67 +/- 50

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Lung cancer

34:

biospecimen:Urine

concentration_value:466

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Not Specified

patient_information:Molybdenum cofactor deficiency

35:

biospecimen:Urine

concentration_value:1261

concentration_units:umol/mmol creatinine

patient_age:Newborn (0-30 days old)

patient_sex:Not Specified

patient_information:Molybdenum cofactor deficiency

36:

biospecimen:Urine

concentration_value:280000000

concentration_units:umol/mmol creatinine

patient_age:Children (1-13 years old)

patient_sex:Female

patient_information:Sulfite oxidase deficiency

37:

biospecimen:Urine

concentration_value:121.6 +/- 3.5

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency

38:

biospecimen:Urine

concentration_value:72.9 +/- 10.7

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Propionic acidemia

39:

biospecimen:Urine

concentration_value:98.3 +/- 7.1

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Maple syrup urine disease

40:

biospecimen:Urine

concentration_value:49.4 +/- 1.4

concentration_units:umol/mmol creatinine

patient_age:Adult (>18 years old)

patient_sex:Both

patient_information:Aminoaciduria

疾病参考
1:

name:Heart failure

omim_id:

[1]:

reference_text:Norrelund H, Wiggers H, Halbirk M, Frystyk J, Flyvbjerg A, Botker HE, Schmitz O, Jorgensen JO, Christiansen JS, Moller N: Abnormalities of whole body protein turnover, muscle metabolism and levels of metabolic hormones in patients with chronic heart failure. J Intern Med. 2006 Jul;260(1):11-21.

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[2]:

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

pubmed_id:

19:

name:Maple syrup urine disease

omim_id:248600

[1]:

reference_text:Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20.

pubmed_id:12101068

[2]:

reference_text:Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U: Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease. Clin Chem. 1999 Oct;45(10):1734-40.

pubmed_id:10508118

[3]:

reference_text:Schadewaldt P, Hammen HW, Ott AC, Wendel U: Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease. J Inherit Metab Dis. 1999 Aug;22(6):706-22.

pubmed_id:10472531

[4]:

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

[5]:

reference_text:Kumps A, Duez P, Mardens Y: Metabolic, nutritional, iatrogenic, and artifactual sources of urinary organic acids: a comprehensive table. Clin Chem. 2002 May;48(5):708-17.

pubmed_id:11978597

[6]:

reference_text:Podebrad F, Heil M, Reichert S, Mosandl A, Sewell AC, Bohles H: 4,5-dimethyl-3-hydroxy-2[5H]-furanone (sotolone)--the odour of maple syrup urine disease. J Inherit Metab Dis. 1999 Apr;22(2):107-14.

pubmed_id:10234605

[7]:

reference_text:Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9.

pubmed_id:6422161

[8]:

reference_text:Yunus ZM, Kamaludin DA, Mamat M, Choy YS, Ngu L: Clinical and biochemical profiles of maple syrup urine disease in malaysian children. JIMD Rep. 2012;5:99-107. doi: 10.1007/8904_2011_105. Epub 2011 Dec 11.

pubmed_id:23430924

[9]:

reference_text:Barschak AG, Marchesan C, Sitta A, Deon M, Giugliani R, Wajner M, Vargas CR: Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem. 2008 Mar;41(4-5):317-24. Epub 2007 Dec 5.

pubmed_id:18088602

[10]:

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

pubmed_id:

[11]:

reference_text:Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404

pubmed_id:

20:

name:Eosinophilic esophagitis

omim_id:610247

[1]:

reference_text:Mordechai, Hien, and David S. Wishart

pubmed_id:

21:

name:Molybdenum cofactor deficiency

omim_id:

[1]:

reference_text:Wevers RA, Engelke UF, Moolenaar SH, Brautigam C, de Jong JG, Duran R, de Abreu RA, van Gennip AH: 1H-NMR spectroscopy of body fluids: inborn errors of purine and pyrimidine metabolism. Clin Chem. 1999 Apr;45(4):539-48.

pubmed_id:10102915

[2]:

reference_text:Sass JO, Kishikawa M, Puttinger R, Reiss J, Erwa W, Shimizu A, Sperl W: Hypohomocysteinaemia and highly increased proportion of S-sulfonated plasma transthyretin in molybdenum cofactor deficiency. J Inherit Metab Dis. 2003;26(1):80-2.

pubmed_id:12872846

[3]:

reference_text:Zaki MS, Selim L, El-Bassyouni HT, Issa MY, Mahmoud I, Ismail S, Girgis M, Sadek AA, Gleeson JG, Abdel Hamid MS: Molybdenum cofactor and isolated sulphite oxidase deficiencies: Clinical and molecular spectrum among Egyptian patients. Eur J Paediatr Neurol. 2016 Sep;20(5):714-22. doi: 10.1016/j.ejpn.2016.05.011. Epub 2016 May 30.

pubmed_id:27289259

[4]:

reference_text:van Gennip AH, Abeling NG, Stroomer AE, Overmars H, Bakker HD: The detection of molybdenum cofactor deficiency: clinical symptomatology and urinary metabolite profile. J Inherit Metab Dis. 1994;17(1):142-5.

pubmed_id:8051926

[5]:

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

pubmed_id:

KEGG数据库编号
C00245
DrugBank数据库编号
DB01956
foodb数据库编号
FDB003191
ChemSpider数据库编号
1091
公共化学化合物编号
1123
PDB数据库编号
生物利益的化学实体数据库编号
15891
knapsack数据库编号
C00048188
维基百科编号
Taurine
代谢途径的数据库编号
TAURINE
苯酚资源管理器化合物数据库编号
比格数据库编号
34373
梅林编号
31
虚拟机身份证编号
TAUR
fbonto数据库编号
综合参考
Hu, Libo; Zhu, Hui; Du, Da-Ming; Xu, Jiaxi. Efficient synthesis of taurine and structurally diverse substituted taurines from aziridines. Journal of Organic Chemistry (2007), 72(12), 4543-4546.
一般参考
1:

protein_accession:HMDBP00549

name:Bile acid-CoA:amino acid N-acyltransferase

uniprot_id:Q14032

gene_name:BAAT

protein_type:Enzyme

2:

protein_accession:HMDBP00702

name:Glutamate decarboxylase 2

uniprot_id:Q05329

gene_name:GAD2

protein_type:Enzyme

3:

protein_accession:HMDBP00703

name:Glutamate decarboxylase 1

uniprot_id:Q99259

gene_name:GAD1

protein_type:Enzyme

4:

protein_accession:HMDBP00730

name:Gamma-glutamyltranspeptidase 1

uniprot_id:P19440

gene_name:GGT1

protein_type:Enzyme

5:

protein_accession:HMDBP00731

name:Gamma-glutamyltransferase 7

uniprot_id:Q9UJ14

gene_name:GGT7

protein_type:Enzyme

6:

protein_accession:HMDBP00936

name:Cysteine sulfinic acid decarboxylase

uniprot_id:Q9Y600

gene_name:CSAD

protein_type:Enzyme

7:

protein_accession:HMDBP02887

name:Sodium- and chloride-dependent taurine transporter

uniprot_id:P31641

gene_name:SLC6A6

protein_type:Unknown

8:

protein_accession:HMDBP07358

name:Gamma-glutamyltransferase 6

uniprot_id:Q6P531

gene_name:GGT6

protein_type:Enzyme

9:

protein_accession:HMDBP08216

name:Glycine receptor subunit alpha-1

uniprot_id:P23415

gene_name:GLRA1

protein_type:Unknown

10:

protein_accession:HMDBP09342

name:GGT7 protein

uniprot_id:A0PJJ9

gene_name:GGT7

protein_type:Enzyme

11:

protein_accession:HMDBP09343

name:Gamma-glutamyltransferase 5

uniprot_id:P36269

gene_name:GGT5

protein_type:Enzyme

蛋白质结合
1:

reference_text:Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762.

pubmed_id:19212411

2:

reference_text:Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50.

pubmed_id:12834252

3:

reference_text:Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7.

pubmed_id:6198473

4:

reference_text:Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14.

pubmed_id:15911239

5:

reference_text:Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6.

pubmed_id:12297216

6:

reference_text:Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24.

pubmed_id:14992292

7:

reference_text:Khan SA, Cox IJ, Hamilton G, Thomas HC, Taylor-Robinson SD: In vivo and in vitro nuclear magnetic resonance spectroscopy as a tool for investigating hepatobiliary disease: a review of H and P MRS applications. Liver Int. 2005 Apr;25(2):273-81.

pubmed_id:15780050

8:

reference_text:Vinton NE, Laidlaw SA, Ament ME, Kopple JD: Taurine concentrations in plasma, blood cells, and urine of children undergoing long-term total parenteral nutrition. Pediatr Res. 1987 Apr;21(4):399-403.

pubmed_id:3106924

9:

reference_text:Gonzalez-Quevedo A, Obregon F, Fernandez R, Santiesteban R, Serrano C, Lima L: Amino acid levels and ratios in serum and cerebrospinal fluid of patients with optic neuropathy in Cuba. Nutr Neurosci. 2001;4(1):51-62.

pubmed_id:11842876

10:

reference_text:Schneider SM, Joly F, Gehrardt MF, Badran AM, Myara A, Thuillier F, Coudray-Lucas C, Cynober L, Trivin F, Messing B: Taurine status and response to intravenous taurine supplementation in adults with short-bowel syndrome undergoing long-term parenteral nutrition: a pilot study. Br J Nutr. 2006 Aug;96(2):365-70.

pubmed_id:16923232

11:

reference_text:McCarty MF: Complementary vascular-protective actions of magnesium and taurine: a rationale for magnesium taurate. Med Hypotheses. 1996 Feb;46(2):89-100.

pubmed_id:8692051

12:

reference_text:Kopple JD, Vinton NE, Laidlaw SA, Ament ME: Effect of intravenous taurine supplementation on plasma, blood cell, and urine taurine concentrations in adults undergoing long-term parenteral nutrition. Am J Clin Nutr. 1990 Nov;52(5):846-53.

pubmed_id:2122710

13:

reference_text:McMahon GP, O'Kennedy R, Kelly MT: High-performance liquid chromatographic determination of taurine in human plasma using pre-column extraction and derivatization. J Pharm Biomed Anal. 1996 Jun;14(8-10):1287-94.

pubmed_id:8818047

14:

reference_text:Stover JF, Morganti-Kosmann MC, Lenzlinger PM, Stocker R, Kempski OS, Kossmann T: Glutamate and taurine are increased in ventricular cerebrospinal fluid of severely brain-injured patients. J Neurotrauma. 1999 Feb;16(2):135-42.

pubmed_id:10098958

15:

reference_text:Learn DB, Fried VA, Thomas EL: Taurine and hypotaurine content of human leukocytes. J Leukoc Biol. 1990 Aug;48(2):174-82.

pubmed_id:2370482

16:

reference_text:Miglis M, Wilder D, Reid T, Bakaltcheva I: Effect of taurine on platelets and the plasma coagulation system. Platelets. 2002 Feb;13(1):5-10.

pubmed_id:11918831

17:

reference_text:Axelson M, Ellis E, Mork B, Garmark K, Abrahamsson A, Bjorkhem I, Ericzon BG, Einarsson C: Bile acid synthesis in cultured human hepatocytes: support for an alternative biosynthetic pathway to cholic acid. Hepatology. 2000 Jun;31(6):1305-12.

pubmed_id:10827156

18:

reference_text:Hu S, Zhao X, Yin S, Meng J: [A study on the mechanism of taurine postponing the aging process of human fetal brain neural cells]. Wei Sheng Yan Jiu. 1997 Mar;26(2):98-101.

pubmed_id:10325611

19:

reference_text:Goodman HO, Shihabi Z, Oles KS: Antiepileptic drugs and plasma and platelet taurine in epilepsy. Epilepsia. 1989 Mar-Apr;30(2):201-7.

pubmed_id:2494044

20:

reference_text:Sturman JA, Messing JM, Rossi SS, Hofmann AF, Neuringer MD: Tissue taurine content and conjugated bile acid composition of rhesus monkey infants fed a human infant soy-protein formula with or without taurine supplementation for 3 months. Neurochem Res. 1988 Apr;13(4):311-6.

pubmed_id:3393260

21:

reference_text:Gonzalez-Quevedo A, Obregon F, Santiesteban Freixas R, Fernandez R, Lima L: [Amino acids as biochemical markers in epidemic and endemic optic neuropathies]. Rev Cubana Med Trop. 1998;50 Suppl:241-4.

pubmed_id:10349454

22:

reference_text:Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043.

pubmed_id:32033212

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