3-hydroxyisobutyrate dehydrogenase
3-hydroxyisobutyrate dehydrogenase | |||||||||
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Identifiers | |||||||||
ExPASy | NiceZyme view | ||||||||
KEGG | KEGG entry | ||||||||
MetaCyc | metabolic pathway | ||||||||
PRIAM | profile | ||||||||
PDB structures | RCSB PDB PDBe PDBsum | ||||||||
Gene Ontology | AmiGO / QuickGO | ||||||||
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HIBADH | |||
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Gene ontology | |||
Molecular function | |||
Cellular component | |||
Biological process | |||
Sources:Amigo / QuickGO |
Ensembl | |||||||||
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UniProt | |||||||||
RefSeq (mRNA) | |||||||||
RefSeq (protein) | |||||||||
Location (UCSC) | Chr 7: 27.53 – 27.66 Mb | Chr 6: 52.52 – 52.62 Mb | |||||||
PubMed search | [3] | [4] |
View/Edit Human | View/Edit Mouse |
In
enzymology, a 3-hydroxyisobutyrate dehydrogenase (EC 1.1.1.31) also known as β-hydroxyisobutyrate dehydrogenase or 3-hydroxyisobutyrate dehydrogenase, mitochondrial (HIBADH) is an enzyme[5] that in humans is encoded by the HIBADH gene.[6]
3-Hydroxyisobutyrate dehydrogenase catalyzes the chemical reaction:
- 3-hydroxy-2-methylpropanoate + NAD+ 2-methyl-3-oxopropanoate + NADH + H+
Thus, the two
.This enzyme belongs to the family of
valine, leucine and isoleucine degradation
.
Function
3-hydroxyisobutyrate dehydrogenase is a tetrameric mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidation of 3-hydroxyisobutyrate, an intermediate of valine catabolism, to methylmalonate semialdehyde.[6]
Structural studies
As of late 2007, five
structures have been solved for this class of enzymes, with PDB accession codes 1WP4, 2CVZ, 2GF2, 2H78, and 2I9P
.
References
- ^ a b c GRCh38: Ensembl release 89: ENSG00000106049 - Ensembl, May 2017
- ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000029776 - Ensembl, May 2017
- ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- PMID 13416257.
- ^ a b "Entrez Gene: HIBADH 3-hydroxyisobutyrate dehydrogenase".
Further reading
- Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. PMID 15489334.
- Hillier LW, Fulton RS, Fulton LA, et al. (2003). "The DNA sequence of human chromosome 7". Nature. 424 (6945): 157–64. PMID 12853948.
- Scherer SW, Cheung J, MacDonald JR, et al. (2003). "Human chromosome 7: DNA sequence and biology". Science. 300 (5620): 767–72. PMID 12690205.
- Mammalian Gene Collection Program Team, Strausberg RL, Feingold EA, Grouse LH, et al. (2002). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". PMID 12477932.
- Sanger Centre T, Washington University Genome Sequencing Cente T (1999). "Toward a complete human genome sequence". Genome Res. 8 (11): 1097–108. PMID 9847074.
- Hughes GJ, Frutiger S, Paquet N, et al. (1994). "Human liver protein map: update 1993". Electrophoresis. 14 (11): 1216–22. S2CID 33424554.
- Rougraff PM, Paxton R, Kuntz MJ, et al. (1988). "Purification and characterization of 3-hydroxyisobutyrate dehydrogenase from rabbit liver". J. Biol. Chem. 263 (1): 327–31. PMID 3335502.
- Rougraff PM, Zhang B, Kuntz MJ, et al. (1989). "Cloning and sequence analysis of a cDNA for 3-hydroxyisobutyrate dehydrogenase. Evidence for its evolutionary relationship to other pyridine nucleotide-dependent dehydrogenases". J. Biol. Chem. 264 (10): 5899–903. PMID 2647728.
External links
- Human HIBADH genome location and HIBADH gene details page in the UCSC Genome Browser.
- PDBe-KB provides an overview of all the structure information available in the PDB for Human 3-hydroxyisobutyrate dehydrogenase, mitochondrial