版本

5.0

创建日期

2005-11-16 15:48:42 UTC

更新日期

2021-09-14 15:20:01 UTC

登录号

HMDB0000023

地位

quantified

名称

(S)-3-Hydroxyisobutyric acid

描述

(S)-3-Hydroxyisobutyric acid (3-HIBA) (CAS: 2068-83-9) is an organic acid. 3-HIBA is an intermediate in L-valine metabolism. 3-HIBA plays an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria (OMIM: 236795) and methylmalonic semialdehyde dehydrogenase deficiency (OMIM: 603178). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. 3-Hydroxyisobutyric aciduria is caused by a 3-hydroxyisobutyryl-CoA dehydrogenase deficiency (PMID: 18329219). The severity of this disease varies from case to case. Most patients exhibit dysmorphic features, such as a small triangular face, a long philtrum, low set ears, and micrognathia (PMID: 10686279). Lactic acidemia is also found in the affected patients, indicating that mitochondrial dysfunction is involved. 3-HIBA appears to specifically inhibit the function of the respiratory chain complex I-III and mitochondrial creatine kinase (PMID: 18329219). BioTransformer predicts that 3-HIBA is a product of 2-methylpropanoic acid metabolism via a hydroxylation-of-terminal-methyl reaction catalyzed by CYP2B6 and CYP2E1 enzymes (PMID: 30612223).

同义词

1:(S)-3-Hydroxy-2-methylpropanoic acid

---

2:(S)-3-Hydroxy-2-methylpropionic acid

---

3:(S)-3-Hydroxy-2-methylpropanoate

---

4:(S)-3-Hydroxy-2-methylpropionate

---

5:(S)-3-Hydroxyisobutyrate

---

6:2-Methyl-L-(+)-hydracrylate

---

7:2-Methyl-L-(+)-hydracrylic acid

---

8:3-Hydroxy(iso)butyric acid

---

9:3-Hydroxy-2-methyl-(S)-propanoate

---

10:3-Hydroxy-2-methyl-(S)-propanoic acid

---

11:3-Hydroxy-2-methylpropanoate

---

12:3-Hydroxy-2-methylpropanoic acid

---

13:3-Hydroxy-isobutyrate

---

14:3-Hydroxyisobutyrate

---

15:3-Hydroxyisobutyric acid

---

16:(2S)-3-Hydroxy-2-methylpropanoic acid

---

17:(2S)-3-Hydroxy-2-methylpropionic acid

---

18:(S)-beta-Hydroxyisobutyric acid

---

19:(S)-Β-hydroxyisobutyric acid

---

20:(±)-3-hydroxy-2-methylpropanoic acid

---

21:(±)-3-hydroxy-2-methylpropionic acid

---

22:2-(Hydroxymethyl)propanoic acid

---

23:2-(Hydroxymethyl)propionic acid

---

24:2-Methyl-3-hydroxypropanoic acid

---

25:2-Methyl-3-hydroxypropionic acid

---

26:3-HIBA

---

27:3-Hydroxy-2-methylpropionic acid

---

28:DL-3-Hydroxyisobutyric acid

---

29:L-(+)-beta-Hydroxyisobutyric acid

---

30:L-(+)-Β-hydroxyisobutyric acid

---

31:beta-Hydroxyisobutyric acid

---

32:Β-hydroxyisobutyric acid

---

33:3-Hydroxy-2-isobutyrate

---

34:3-Hydroxy-2-isobutyric acid

---

35:(S)-3-Hydroxyisobutyric acid

---

化学式

C4H8O3

平均分子量

104.1045

等位分子量

104.047344122

化学名称

(2S)-3-hydroxy-2-methylpropanoic acid

传统名称

(S)-3-hydroxyisobutyric acid

CAS 登记号

26543-05-5

代谢物结构字符串

C[C@@H](CO)C(O)=O

inchi标识符

InChI=1S/C4H8O3/c1-3(2-5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1

印记键

DBXBTMSZEOQQDU-VKHMYHEASA-N

分类学

description: belongs to the class of organic compounds known as beta hydroxy acids and derivatives. Beta hydroxy acids and derivatives are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom.

---

description:Beta hydroxy acids and derivatives

---

kingdom:Organic compounds

---

super_class:Organic acids and derivatives

---

class:Hydroxy acids and derivatives

---

sub_class:Beta hydroxy acids and derivatives

---

molecular_framework:Aliphatic acyclic compounds

---

1:Carbonyl compounds

---

2:Carboxylic acids

---

3:Hydrocarbon derivatives

---

4:Monocarboxylic acids and derivatives

---

5:Organic oxides

---

6:Primary alcohols

---

7:Alcohol

---

8:Aliphatic acyclic compound

---

9:Beta-hydroxy acid

---

10:Carbonyl group

---

11:Carboxylic acid

---

12:Carboxylic acid derivative

---

13:Hydrocarbon derivative

---

14:Monocarboxylic acid or derivatives

---

15:Organic oxide

---

16:Organic oxygen compound

---

17:Organooxygen compound

---

18:Primary alcohol

---

19:3-hydroxyisobutyric 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: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: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: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:Biological location

---

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

---

parent_id:7724

---

*level:2

---

type:parent

---

term:Biofluid and excreta

---

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

---

parent_id:7725

---

**level:3

---

type:parent

---

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:Urine

---

definition:Excretion in liquid state processed by the kidney.

---

parent_id:7731

---

***level:4

---

type:child

---

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: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: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

---

状态

Solid

实验性质

预测性质

1:

---

kind:logp

---

value:-0.47

---

source:ALOGPS

---

2:

---

kind:logs

---

value:0.74

---

source:ALOGPS

---

3:

---

kind:solubility

---

value:571 g/L

---

source:ALOGPS

---

4:

---

kind:logp

---

value:-0.26

---

source:ChemAxon

---

5:

---

kind:pka_strongest_acidic

---

value:4.37

---

source:ChemAxon

---

6:

---

kind:pka_strongest_basic

---

value:-2.7

---

source:ChemAxon

---

7:

---

kind:iupac

---

value:(2S)-3-hydroxy-2-methylpropanoic acid

---

source:ChemAxon

---

8:

---

kind:average_mass

---

value:104.1045

---

source:ChemAxon

---

9:

---

kind:mono_mass

---

value:104.047344122

---

source:ChemAxon

---

10:

---

kind:smiles

---

value:C[C@@H](CO)C(O)=O

---

source:ChemAxon

---

11:

---

kind:formula

---

value:C4H8O3

---

source:ChemAxon

---

12:

---

kind:inchi

---

value:InChI=1S/C4H8O3/c1-3(2-5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1

---

source:ChemAxon

---

13:

---

kind:inchikey

---

value:DBXBTMSZEOQQDU-VKHMYHEASA-N

---

source:ChemAxon

---

14:

---

kind:polar_surface_area

---

value:57.53

---

source:ChemAxon

---

15:

---

kind:refractivity

---

value:23.62

---

source:ChemAxon

---

16:

---

kind:polarizability

---

value:9.98

---

source:ChemAxon

---

17:

---

kind:rotatable_bond_count

---

value:2

---

source:ChemAxon

---

18:

---

kind:acceptor_count

---

value:3

---

source:ChemAxon

---

19:

---

kind:donor_count

---

value:2

---

source:ChemAxon

---

20:

---

kind:physiological_charge

---

value:-1

---

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

---

光谱

生物学性质

1:

---

cellular:(1):Cytoplasm

---

cellular:(2):Mitochondria

---

2:

---

biospecimen:(1):Blood

---

biospecimen:(2):Cerebrospinal Fluid (CSF)

---

biospecimen:(3):Feces

---

biospecimen:(4):Saliva

---

biospecimen:(5):Urine

---

3:

---

4:

---

[1]:

---

name:2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency

---

smpdb_id:SMP00137

---

kegg_map_id:

---

[2]:

---

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

---

smpdb_id:SMP00138

---

kegg_map_id:

---

[3]:

---

name:3-hydroxyisobutyric acid dehydrogenase deficiency

---

smpdb_id:SMP00521

---

kegg_map_id:

---

[4]:

---

name:3-hydroxyisobutyric aciduria

---

smpdb_id:SMP00522

---

kegg_map_id:

---

[5]:

---

name:3-Methylcrotonyl Coa Carboxylase Deficiency Type I

---

smpdb_id:SMP00237

---

kegg_map_id:

---

[6]:

---

name:3-Methylglutaconic Aciduria Type I

---

smpdb_id:SMP00139

---

kegg_map_id:

---

[7]:

---

name:3-Methylglutaconic Aciduria Type III

---

smpdb_id:SMP00140

---

kegg_map_id:

---

[8]:

---

name:3-Methylglutaconic Aciduria Type IV

---

smpdb_id:SMP00141

---

kegg_map_id:

---

[9]:

---

name:Beta-Ketothiolase Deficiency

---

smpdb_id:SMP00173

---

kegg_map_id:

---

[10]:

---

name:Isobutyryl-coa dehydrogenase deficiency

---

smpdb_id:SMP00523

---

kegg_map_id:

---

[11]:

---

name:Isovaleric acidemia

---

smpdb_id:SMP00524

---

kegg_map_id:

---

[12]:

---

name:Isovaleric Aciduria

---

smpdb_id:SMP00238

---

kegg_map_id:

---

[13]:

---

name:Maple Syrup Urine Disease

---

smpdb_id:SMP00199

---

kegg_map_id:

---

[14]:

---

name:Methylmalonate Semialdehyde Dehydrogenase Deficiency

---

smpdb_id:SMP00384

---

kegg_map_id:

---

[15]:

---

name:Methylmalonic Aciduria

---

smpdb_id:SMP00200

---

kegg_map_id:

---

[16]:

---

name:Propionic Acidemia

---

smpdb_id:SMP00236

---

kegg_map_id:

---

[17]:

---

name:Valine, leucine and isoleucine degradation

---

smpdb_id:

---

kegg_map_id:map00280

---

正常浓度

1:

---

biospecimen:Blood

---

concentration_value:21.0 +/- 2.0

---

concentration_units:uM

---

subject_age:Adult (>18 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

2:

---

biospecimen:Blood

---

concentration_value:20.0 (4.0-48.0)

---

concentration_units:uM

---

subject_age:Children (1-13 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

3:

---

biospecimen:Cerebrospinal Fluid (CSF)

---

concentration_value:18.0 +/- 18.0 (0 - 36)

---

concentration_units:uM

---

subject_age:Adult (>18 years old)

---

subject_sex:Not Specified

---

subject_condition:Normal

---

4:

---

biospecimen:Feces

---

concentration_value:

---

concentration_units:

---

subject_age:Adult (>18 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

5:

---

biospecimen:Saliva

---

concentration_value:1.23 +/- 0.71

---

concentration_units:uM

---

subject_age:Adult (>18 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

6:

---

biospecimen:Urine

---

concentration_value:29.0 (11.8-59.8)

---

concentration_units:umol/mmol creatinine

---

subject_age:Adult (>18 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

7:

---

biospecimen:Urine

---

concentration_value:10-17.0

---

concentration_units:umol/mmol creatinine

---

subject_age:Infant (0-1 year old)

---

subject_sex:Male

---

subject_condition:Normal

---

8:

---

biospecimen:Urine

---

concentration_value:10.0-15.9

---

concentration_units:umol/mmol creatinine

---

subject_age:Infant (0-1 year old)

---

subject_sex:Female

---

subject_condition:Normal

---

9:

---

biospecimen:Urine

---

concentration_value:6.9-13.4

---

concentration_units:umol/mmol creatinine

---

subject_age:Infant (0-1 year old)

---

subject_sex:Female

---

subject_condition:Normal

---

10:

---

biospecimen:Urine

---

concentration_value:8.1-18

---

concentration_units:umol/mmol creatinine

---

subject_age:Infant (0-1 year old)

---

subject_sex:Male

---

subject_condition:Normal

---

11:

---

biospecimen:Urine

---

concentration_value:13.39 +/- 6.588

---

concentration_units:umol/mmol creatinine

---

subject_age:Children (1 - 13 years old)

---

subject_sex:Not Specified

---

subject_condition:Normal

---

12:

---

biospecimen:Urine

---

concentration_value:13.0 (2.0-24.0)

---

concentration_units:umol/mmol creatinine

---

subject_age:Children (1-13 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

13:

---

biospecimen:Urine

---

concentration_value:11 (4.1-19)

---

concentration_units:umol/mmol creatinine

---

subject_age:Adult (>18 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

14:

---

biospecimen:Urine

---

concentration_value:<95

---

concentration_units:umol/mmol creatinine

---

subject_age:Children (1 - 18 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

15:

---

biospecimen:Urine

---

concentration_value:14.5 (5.5-28.7)

---

concentration_units:umol/mmol creatinine

---

subject_age:Newborn (0-30 days old)

---

subject_sex:Both

---

subject_condition:Normal

---

16:

---

biospecimen:Urine

---

concentration_value:8.5 (4.9-17.1)

---

concentration_units:umol/mmol creatinine

---

subject_age:Children (1-13 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

17:

---

biospecimen:Urine

---

concentration_value:7.8 (3.8-25.1)

---

concentration_units:umol/mmol creatinine

---

subject_age:Adolescent (13-18 years old)

---

subject_sex:Both

---

subject_condition:Normal

---

18:

---

biospecimen:Urine

---

concentration_value:25.4 (10.2 -33.5)

---

concentration_units:umol/mmol creatinine

---

subject_age:Infant (0-1 year old)

---

subject_sex:Both

---

subject_condition:Normal

---

异常浓度

1:

---

biospecimen:Blood

---

concentration_value:38.0 +/- 5.0

---

concentration_units:uM

---

patient_age:Adult (>18 years old)

---

patient_sex:Both

---

patient_information:Diabetes

---

2:

---

biospecimen:Feces

---

concentration_value:

---

concentration_units:

---

patient_age:Adult (>18 years old)

---

patient_sex:Both

---

patient_information:Colorectal cancer

---

3:

---

biospecimen:Feces

---

concentration_value:

---

concentration_units:

---

patient_age:Adult (>18 years old)

---

patient_sex:Both

---

patient_information:Colorectal cancer

---

4:

---

biospecimen:Urine

---

concentration_value:12.772 +/- 10.179

---

concentration_units:umol/mmol creatinine

---

patient_age:Children (1 - 13 years old)

---

patient_sex:Not Specified

---

patient_information:Eosinophilic esophagitis

---

5:

---

biospecimen:Urine

---

concentration_value:17.5 (2.0-33.0)

---

concentration_units:umol/mmol creatinine

---

patient_age:Adult (>18 years old)

---

patient_sex:Both

---

patient_information:3-Hydroxyisobutyric aciduria

---

6:

---

biospecimen:Urine

---

concentration_value:225.0 (60.0-390.0)

---

concentration_units:umol/mmol creatinine

---

patient_age:Children (1-13 years old)

---

patient_sex:Both

---

patient_information:3-Hydroxyisobutyric Aciduria

---

疾病参考

1:

---

name:Diabetes mellitus type 2

---

omim_id:125853

---

[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: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.

---

pubmed_id:11887176

---

[3]:

---

reference_text:Dungan KM, Buse JB, Largay J, Kelly MM, Button EA, Kato S, Wittlin S: 1,5-anhydroglucitol and postprandial hyperglycemia as measured by continuous glucose monitoring system in moderately controlled patients with diabetes. Diabetes Care. 2006 Jun;29(6):1214-9.

---

pubmed_id:16731998

---

[4]:

---

reference_text:Sheikh-Ali M, Karon BS, Basu A, Kudva YC, Muller LA, Xu J, Schwenk WF, Miles JM: Can serum beta-hydroxybutyrate be used to diagnose diabetic ketoacidosis? Diabetes Care. 2008 Apr;31(4):643-7. doi: 10.2337/dc07-1683. Epub 2008 Jan 9.

---

pubmed_id:18184896

---

[5]:

---

reference_text:Tasker RC, Lutman D, Peters MJ: Hyperventilation in severe diabetic ketoacidosis. Pediatr Crit Care Med. 2005 Jul;6(4):405-11.

---

pubmed_id:15982426

---

[6]:

---

reference_text:Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32.

---

pubmed_id:6321058

---

[7]:

---

reference_text:Wahl HG, Hoffmann A, Luft D, Liebich HM: Analysis of volatile organic compounds in human urine by headspace gas chromatography-mass spectrometry with a multipurpose sampler. J Chromatogr A. 1999 Jun 25;847(1-2):117-25.

---

pubmed_id:10431355

---

[8]:

---

reference_text:Hoppel CL, Genuth SM: Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis. Am J Physiol. 1982 Aug;243(2):E168-72.

---

pubmed_id:6810706

---

[9]:

---

reference_text:Zhang AQ, Mitchell SC, Smith RL: Dimethylamine in human urine. Clin Chim Acta. 1995 Jan 16;233(1-2):81-8.

---

pubmed_id:7758205

---

[10]:

---

reference_text:Stickle D, Turk J: A kinetic mass balance model for 1,5-anhydroglucitol: applications to monitoring of glycemic control. Am J Physiol. 1997 Oct;273(4 Pt 1):E821-30.

---

pubmed_id:9357814

---

[11]:

---

reference_text:del Carmen Crespillo M, Olveira G, de Adana MS, Rojo-Martinez G, Garcia-Aleman J, Olvera P, Soriguer F, Munoz A: Metabolic effects of an enteral nutrition formula for diabetes: comparison with standard formulas in patients with type 1 diabetes. Clin Nutr. 2003 Oct;22(5):483-7.

---

pubmed_id:14512036

---

[12]:

---

reference_text:Blomqvist G, Alvarsson M, Grill V, Von Heijne G, Ingvar M, Thorell JO, Stone-Elander S, Widen L, Ekberg K: Effect of acute hyperketonemia on the cerebral uptake of ketone bodies in nondiabetic subjects and IDDM patients. Am J Physiol Endocrinol Metab. 2002 Jul;283(1):E20-8.

---

pubmed_id:12067838

---

[13]:

---

reference_text:Stupar J, Vrtovec M, Dolinsek F: Longitudinal hair chromium profiles of elderly subjects with normal glucose tolerance and type 2 diabetes mellitus. Metabolism. 2007 Jan;56(1):94-104.

---

pubmed_id:17161231

---

[14]:

---

reference_text:Shin CS, Moon BS, Park KS, Kim SY, Park SJ, Chung MH, Lee HK: Serum 8-hydroxy-guanine levels are increased in diabetic patients. Diabetes Care. 2001 Apr;24(4):733-7.

---

pubmed_id:11315839

---

[15]:

---

reference_text:Nguyen TM, Rodriguez LM, Mason KJ, Heptulla RA: Serum 1,5-anhydroglucitol (Glycomark) levels in children with and without type 1 diabetes mellitus. Pediatr Diabetes. 2007 Aug;8(4):214-9.

---

pubmed_id:17659063

---

[16]:

---

reference_text:Guevara-Aguirre J, Guevara-Aguirre M, Saavedra J, Bernstein G, Rosenbloom AL: Comparison of oral insulin spray and subcutaneous regular insulin at mealtime in type 1 diabetes. Diabetes Technol Ther. 2007 Aug;9(4):372-6.

---

pubmed_id:17705693

---

[17]:

---

reference_text:McLellan AC, Phillips SA, Thornalley PJ: Fluorimetric assay of D-lactate. Anal Biochem. 1992 Oct;206(1):12-6.

---

pubmed_id:1456422

---

[18]:

---

reference_text:Giussi GA, Ballejo G, Benedetti WL: Estriol serum levels, neonatal vitality and stromal villous edema in diabetic pregnancies. Acta Diabetol Lat. 1979 Apr-Jun;16(2):105-10.

---

pubmed_id:484160

---

[19]:

---

reference_text:Ostlund RE Jr, McGill JB, Herskowitz I, Kipnis DM, Santiago JV, Sherman WR: D-chiro-inositol metabolism in diabetes mellitus. Proc Natl Acad Sci U S A. 1993 Nov 1;90(21):9988-92.

---

pubmed_id:8234346

---

[20]:

---

reference_text:Greco AV, Mingrone G, Capristo E, Benedetti G, De Gaetano A, Gasbarrini G: The metabolic effect of dodecanedioic acid infusion in non-insulin-dependent diabetic patients. Nutrition. 1998 Apr;14(4):351-7.

---

pubmed_id:9591306

---

[21]:

---

reference_text:Mine S, Okada Y, Kawahara C, Tabata T, Tanaka Y: Serum hyaluronan concentration as a marker of angiopathy in patients with diabetes mellitus. Endocr J. 2006 Dec;53(6):761-6. Epub 2006 Sep 12.

---

pubmed_id:16966827

---

[22]:

---

reference_text:Liebich HM, Buelow HJ, Kallmayer R: Quantification of endogenous aliphatic alcohols in serum and urine. J Chromatogr. 1982 Apr 30;239:343-9.

---

pubmed_id:7096503

---

[23]:

---

reference_text:Avogaro A, Bier DM: Contribution of 3-hydroxyisobutyrate to the measurement of 3-hydroxybutyrate in human plasma: comparison of enzymatic and gas-liquid chromatography-mass spectrometry assays in normal and in diabetic subjects. J Lipid Res. 1989 Nov;30(11):1811-7.

---

pubmed_id:2614280

---

[24]:

---

reference_text:Khuhawar MY, Zardari LA, Laghari AJ: Capillary gas chromatographic determination of methylglyoxal from serum of diabetic patients by precolumn derivatization with 1,2-diamonopropane. J Chromatogr B Analyt Technol Biomed Life Sci. 2008 Sep 15;873(1):15-9. doi: 10.1016/j.jchromb.2008.04.048. Epub 2008 May 17.

---

pubmed_id:18760976

---

[25]:

---

reference_text:Kawasaki T, Akanuma H, Yamanouchi T: Increased fructose concentrations in blood and urine in patients with diabetes. Diabetes Care. 2002 Feb;25(2):353-7.

---

pubmed_id:11815509

---

[26]:

---

reference_text:Noroozi M, Burns J, Crozier A, Kelly IE, Lean ME: Prediction of dietary flavonol consumption from fasting plasma concentration or urinary excretion. Eur J Clin Nutr. 2000 Feb;54(2):143-9.

---

pubmed_id:10694785

---

[27]:

---

reference_text:Paroni R, Ceriotti F, Galanello R, Battista Leoni G, Panico A, Scurati E, Paleari R, Chemello L, Quaino V, Scaldaferri L, Lapolla A, Mosca A: Performance characteristics and clinical utility of an enzymatic method for the measurement of glycated albumin in plasma. Clin Biochem. 2007 Dec;40(18):1398-405. Epub 2007 Aug 10.

---

pubmed_id:17919531

---

[28]:

---

reference_text:Salek RM, Maguire ML, Bentley E, Rubtsov DV, Hough T, Cheeseman M, Nunez D, Sweatman BC, Haselden JN, Cox RD, Connor SC, Griffin JL: A metabolomic comparison of urinary changes in type 2 diabetes in mouse, rat, and human. Physiol Genomics. 2007 Apr 24;29(2):99-108. Epub 2006 Dec 26.

---

pubmed_id:17190852

---

[29]:

---

reference_text:Galli E, Marchini M, Saba A, Berti S, Tonacchera M, Vitti P, Scanlan TS, Iervasi G, Zucchi R: Detection of 3-iodothyronamine in human patients: a preliminary study. J Clin Endocrinol Metab. 2012 Jan;97(1):E69-74. doi: 10.1210/jc.2011-1115. Epub 2011 Oct 26.

---

pubmed_id:22031514

---

[30]:

---

reference_text:Llorach R, Urpi-Sarda M, Tulipani S, Garcia-Aloy M, Monagas M, Andres-Lacueva C: Metabolomic fingerprint in patients at high risk of cardiovascular disease by cocoa intervention. Mol Nutr Food Res. 2013 Jun;57(6):962-73. doi: 10.1002/mnfr.201200736. Epub 2013 May 2.

---

pubmed_id:23637065

---

[31]:

---

reference_text:World Health Organisation Department of Noncommunicable Disease Surveillance (1999). "Definition, Diagnosis and Classification of Diabetes Mellitus and its Complications"

---

pubmed_id:

---

[32]:

---

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

---

2:

---

name:Colorectal cancer

---

omim_id:114500

---

[1]:

---

reference_text:Ishiwata S, Itoh K, Yamaguchi T, Ishida N, Mizugaki M: Comparison of serum and urinary levels of modified nucleoside, 1-methyladenosine, in cancer patients using a monoclonal antibody-based inhibition ELISA. Tohoku J Exp Med. 1995 May;176(1):61-8.

---

pubmed_id:7482520

---

[2]:

---

reference_text:Monleon D, Morales JM, Barrasa A, Lopez JA, Vazquez C, Celda B: Metabolite profiling of fecal water extracts from human colorectal cancer. NMR Biomed. 2009 Apr;22(3):342-8. doi: 10.1002/nbm.1345.

---

pubmed_id:19006102

---

[3]:

---

reference_text:Weir TL, Manter DK, Sheflin AM, Barnett BA, Heuberger AL, Ryan EP: Stool microbiome and metabolome differences between colorectal cancer patients and healthy adults. PLoS One. 2013 Aug 6;8(8):e70803. doi: 10.1371/journal.pone.0070803. Print 2013.

---

pubmed_id:23940645

---

[4]:

---

reference_text:Phua LC, Chue XP, Koh PK, Cheah PY, Ho HK, Chan EC: Non-invasive fecal metabonomic detection of colorectal cancer. Cancer Biol Ther. 2014 Apr;15(4):389-97. doi: 10.4161/cbt.27625. Epub 2014 Jan 14.

---

pubmed_id:24424155

---

[5]:

---

reference_text:Ritchie SA, Ahiahonu PW, Jayasinghe D, Heath D, Liu J, Lu Y, Jin W, Kavianpour A, Yamazaki Y, Khan AM, Hossain M, Su-Myat KK, Wood PL, Krenitsky K, Takemasa I, Miyake M, Sekimoto M, Monden M, Matsubara H, Nomura F, Goodenowe DB: Reduced levels of hydroxylated, polyunsaturated ultra long-chain fatty acids in the serum of colorectal cancer patients: implications for early screening and detection. BMC Med. 2010 Feb 15;8:13. doi: 10.1186/1741-7015-8-13.

---

pubmed_id:20156336

---

[6]:

---

reference_text:Qiu Y, Cai G, Su M, Chen T, Zheng X, Xu Y, Ni Y, Zhao A, Xu LX, Cai S, Jia W: Serum metabolite profiling of human colorectal cancer using GC-TOFMS and UPLC-QTOFMS. J Proteome Res. 2009 Oct;8(10):4844-50. doi: 10.1021/pr9004162.

---

pubmed_id:19678709

---

[7]:

---

reference_text:Cheng Y, Xie G, Chen T, Qiu Y, Zou X, Zheng M, Tan B, Feng B, Dong T, He P, Zhao L, Zhao A, Xu LX, Zhang Y, Jia W: Distinct urinary metabolic profile of human colorectal cancer. J Proteome Res. 2012 Feb 3;11(2):1354-63. doi: 10.1021/pr201001a. Epub 2011 Dec 28.

---

pubmed_id:22148915

---

[8]:

---

reference_text:Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14.

---

pubmed_id:25105552

---

[9]:

---

reference_text:Ikeda A, Nishiumi S, Shinohara M, Yoshie T, Hatano N, Okuno T, Bamba T, Fukusaki E, Takenawa T, Azuma T, Yoshida M: Serum metabolomics as a novel diagnostic approach for gastrointestinal cancer. Biomed Chromatogr. 2012 May;26(5):548-58. doi: 10.1002/bmc.1671. Epub 2011 Jul 20.

---

pubmed_id:21773981

---

[10]:

---

reference_text:Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18.

---

pubmed_id:25037050

---

[11]:

---

reference_text:Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016.

---

pubmed_id:27015276

---

[12]:

---

reference_text:Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762.

---

pubmed_id:27107423

---

[13]:

---

reference_text:Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016.

---

pubmed_id:27275383

---

[14]:

---

reference_text:Wang X, Wang J, Rao B, Deng L: Gut flora profiling and fecal metabolite composition of colorectal cancer patients and healthy individuals. Exp Ther Med. 2017 Jun;13(6):2848-2854. doi: 10.3892/etm.2017.4367. Epub 2017 Apr 20.

---

pubmed_id:28587349

---

[15]:

---

reference_text:Silke Matysik, Caroline Ivanne Le Roy, Gerhard Liebisch, Sandrine Paule Claus. Metabolomics of fecal samples: A practical consideration. Trends in Food Science & Technology. Vol. 57, Part B, Nov. 2016, p.244-255: http://www.sciencedirect.com/science/article/pii/S0924224416301984

---

3:

---

name:3-Hydroxyisobutyric aciduria

---

omim_id:236795

---

[1]:

---

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

---

pubmed_id:

---

4:

---

name:Eosinophilic esophagitis

---

omim_id:610247

---

[1]:

---

reference_text:Mordechai, Hien, and David S. Wishart

---

pubmed_id:

---

KEGG数据库编号

C06001

DrugBank数据库编号

foodb数据库编号

FDB021877

ChemSpider数据库编号

389707

公共化学化合物编号

440873

PDB数据库编号

生物利益的化学实体数据库编号

37373

knapsack数据库编号

维基百科编号

代谢途径的数据库编号

CPD-12175

苯酚资源管理器化合物数据库编号

比格数据库编号

37034

梅林编号

483

虚拟机身份证编号

3HMP

fbonto数据库编号

综合参考

Robison, Robert S.; Doremus, Michael G.L-(+)-b-Hydroxyisobutyric acid by fermentation. U.S. (1986), 5 pp.

一般参考

1:

---

protein_accession:HMDBP00355

---

name:Sulfotransferase family cytosolic 2B member 1

---

uniprot_id:O00204

---

gene_name:SULT2B1

---

protein_type:Enzyme

---

2:

---

protein_accession:HMDBP00380

---

name:Peroxisomal bifunctional enzyme

---

uniprot_id:Q08426

---

gene_name:EHHADH

---

protein_type:Unknown

---

3:

---

protein_accession:HMDBP00381

---

name:Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial

---

uniprot_id:Q16836

---

gene_name:HADH

---

protein_type:Unknown

---

4:

---

protein_accession:HMDBP00395

---

name:3-hydroxyisobutyrate dehydrogenase, mitochondrial

---

uniprot_id:P31937

---

gene_name:HIBADH

---

protein_type:Unknown

---

5:

---

protein_accession:HMDBP00483

---

name:UDP-glucuronosyltransferase 1-1

---

uniprot_id:P22309

---

gene_name:UGT1A1

---

protein_type:Enzyme

---

6:

---

protein_accession:HMDBP04709

---

name:3-hydroxyisobutyryl-CoA hydrolase, mitochondrial

---

uniprot_id:Q6NVY1

---

gene_name:HIBCH

---

protein_type:Enzyme

---

蛋白质结合

1:

---

reference_text:Yoshida I, Sweetman L, Kulovich S, Nyhan WL, Robinson BH: Effect of lipoic acid in a patient with defective activity of pyruvate dehydrogenase, 2-oxoglutarate dehydrogenase, and branched-chain keto acid dehydrogenase. Pediatr Res. 1990 Jan;27(1):75-9.

---

pubmed_id:2104971

---

2:

---

reference_text:Liebich HM, Dotzauer A, Tetschner B: Gas chromatographic determination of oxo- and hydroxycarboxylic acids in serum and urine of diabetic and normal subjects. J Chromatogr. 1989 May 12;468:157-65.

---

pubmed_id:2732286

---

3:

---

reference_text:Podebrad F, Heil M, Beck T, Mosandl A, Sewell AC, Bohles H: Stereodifferentiation of 3-hydroxyisobutyric- and 3-aminoisobutyric acid in human urine by enantioselective multidimensional capillary gas chromatography-mass spectrometry. Clin Chim Acta. 2000 Feb 25;292(1-2):93-105.

---

pubmed_id:10686279

---

4:

---

reference_text:Chitayat D, Meagher-Villemure K, Mamer OA, O'Gorman A, Hoar DI, Silver K, Scriver CR: Brain dysgenesis and congenital intracerebral calcification associated with 3-hydroxyisobutyric aciduria. J Pediatr. 1992 Jul;121(1):86-9.

---

pubmed_id:1625099

---

5:

---

reference_text:Landaas S: Accumulation of 3-hydroxyisobutyric acid, 2-methyl-3-hydroxybutyric acid and 3-hydroxyisovaleric acid in ketoacidosis. Clin Chim Acta. 1975 Oct 15;64(2):143-54.

---

pubmed_id:126826

---

6:

---

reference_text:Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69.

---

pubmed_id:8412012

---

7:

---

reference_text:Djoumbou-Feunang Y, Fiamoncini J, Gil-de-la-Fuente A, Greiner R, Manach C, Wishart DS: BioTransformer: a comprehensive computational tool for small molecule metabolism prediction and metabolite identification. J Cheminform. 2019 Jan 5;11(1):2. doi: 10.1186/s13321-018-0324-5.

---

pubmed_id:30612223

---

8:

---

reference_text:Viegas CM, da Costa Ferreira G, Schuck PF, Tonin AM, Zanatta A, de Souza Wyse AT, Dutra-Filho CS, Wannmacher CM, Wajner M: Evidence that 3-hydroxyisobutyric acid inhibits key enzymes of energy metabolism in cerebral cortex of young rats. Int J Dev Neurosci. 2008 May-Jun;26(3-4):293-9. doi: 10.1016/j.ijdevneu.2008.01.007. Epub 2008 Feb 7.

---

pubmed_id:18329219

---