| InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8) |
| QHKABHOOEWYVLI-UHFFFAOYSA-N |
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Saccharomyces cerevisiae
(NCBI:txid4932)
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See:
PubMed
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Homo sapiens
(NCBI:txid9606)
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See:
PubMed
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Bronsted acid
A molecular entity capable of donating a hydron to an acceptor (Bronsted base).
(via oxoacid )
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human metabolite
Any mammalian metabolite produced during a metabolic reaction in humans (Homo sapiens).
Saccharomyces cerevisiae metabolite
Any fungal metabolite produced during a metabolic reaction in Baker's yeast (Saccharomyces cerevisiae ).
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View more via ChEBI Ontology
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3-methyl-2-oxobutanoic acid
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2-Keto-3-methylbutyric acid
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KEGG COMPOUND
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2-Ketoisovaleric acid
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HMDB
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2-Ketovaline
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KEGG COMPOUND
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2-Oxo-3-methylbutanoic acid
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HMDB
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2-Oxo-3-methylbutyric acid
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HMDB
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2-Oxoisovaleric acid
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HMDB
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3-Methyl-2-oxobutanoate
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KEGG COMPOUND
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3-Methyl-2-oxobutanoic acid
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KEGG COMPOUND
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3-METHYL-2-OXOBUTANOIC ACID
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PDBeChem
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3-Methyl-2-oxobutyric acid
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KEGG COMPOUND
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α-keto-isovaleric acid
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HMDB
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alpha-Ketovaline
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KEGG COMPOUND
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α-oxo-β-methylbutyricacid
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HMDB
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α-oxoisovaleric acid
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HMDB
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Dimethylpyruvic acid
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HMDB
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Isopropylglyoxylic acid
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HMDB
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1744951
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Reaxys Registry Number
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Reaxys
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759-05-7
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CAS Registry Number
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KEGG COMPOUND
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759-05-7
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CAS Registry Number
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ChemIDplus
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Salek RM, Maguire ML, Bentley E, Rubtsov DV, Hough T, Cheeseman M, Nunez D, Sweatman BC, Haselden JN, Cox RD, Connor SC, Griffin JL (2007)
A metabolomic comparison of urinary changes in type 2 diabetes in mouse, rat, and human.
Physiological genomics 29, 99-108 [PubMed:17190852]
[show Abstract]
Type 2 diabetes mellitus is the result of a combination of impaired insulin secretion with reduced insulin sensitivity of target tissues. There are an estimated 150 million affected individuals worldwide, of whom a large proportion remains undiagnosed because of a lack of specific symptoms early in this disorder and inadequate diagnostics. In this study, NMR-based metabolomic analysis in conjunction with multivariate statistics was applied to examine the urinary metabolic changes in two rodent models of type 2 diabetes mellitus as well as unmedicated human sufferers. The db/db mouse and obese Zucker (fa/fa) rat have autosomal recessive defects in the leptin receptor gene, causing type 2 diabetes. 1H-NMR spectra of urine were used in conjunction with uni- and multivariate statistics to identify disease-related metabolic changes in these two animal models and human sufferers. This study demonstrates metabolic similarities between the three species examined, including metabolic responses associated with general systemic stress, changes in the TCA cycle, and perturbations in nucleotide metabolism and in methylamine metabolism. All three species demonstrated profound changes in nucleotide metabolism, including that of N-methylnicotinamide and N-methyl-2-pyridone-5-carboxamide, which may provide unique biomarkers for following type 2 diabetes mellitus progression. | Schauder P, Matthaei D, Henning HV, Scheler F, Langenbeck U (1981)
Blood levels of branched-chain alpha-keto acids in uremia: effect of an oral glucose tolerance test.
Klinische Wochenschrift 59, 845-849 [PubMed:7021997]
[show Abstract]
The effect of an oral glucose tolerance test (oGTT) on serum levels of branched-chain keto acids (BCKA), i.e. alpha-keto-isocaproic acid (KICA), alpha-keto-isovaleric acid (KIVA) and alpha-keto-beta methyl-n-valeric acid (KMVA) as well as on serum insulin, C-peptide and blood glucose levels was determined in uremic patients and in healthy control subjects. In controls, blood levels of KICA, KMVA and KIVA declined significantly following oral administration of 100 glucose. In uremic patients no decline of KICA was observed. The fall of KMVA was diminished, while suppression of KIVA blood levels in response to the oGGT remained unimpaired. Although serum insulin and C-peptide levels in uremic patients were not significantly different from the controls before and throughout the oGTT, six out of eight displayed abnormal glucose tolerance. It is suggested that the response of blood BCKA levels to an oGTT is altered in uremia, an abnormality restricted primarily to KICA and possibly explained by insulin antagonism and/or by insufficient insulin secretion. | Singh S, Willers I, Goedde HW (1977)
Heterogeneity in maple syrup urine disease: aspects of cofactor requirement and complementation in cultured fibroblasts.
Clinical genetics 11, 277-284 [PubMed:192504]
[show Abstract]
Fibroblast strains derived from six patients with maple syrup urine disease have been investigated for their requirements of the cofactors NAD, CoASH, Mg++ and TPP in comparison with 10 normal control strains. The reconstitution of the decarboxylase function of branched chain alpha-keto acid (BCKA) dehydrogenase complex in lysed cells was studied with respect to the substrates alpha-keto-isocaproic acid, alpha-keto-isovaleric acid, and alpha-keto-beta-methylvaleric acid (KIC, KIVA, MEVA). The enzyme activity of all normal control strains for the substrates KIC and KIVA was not reconstituted by TPP + Mg++ alone, but CoASH + NAD could reconstitute the enzyme activity with KIC and KIVA in different degrees. Only two control strains were tested with MEVA as substrate, and these showed in contrast that TPP + Mg++ could partly reconstitute the enzyme activity. In contrast to the relative homogeneity in the reconstitution profiles of normal strains, the five classical and one intermittent MSUD strains showed heterogeneity in cofactor requirements. Complementation analysis using heterokaryons prepared from fibroblasts of four patients with classical MSUD and one patient with intermittent MSUD showed, in contrast to experiments with normal controls, a partial amelioration of the defect in two combinations; it is suggested that the defect in these strains is located at different functional subunits of the multienzyme complex. |
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