3-hydroxyisobutyrate dehydrogenase

HIBADH
Identifiers
Aliases	HIBADH, Hibadh, 6430402H10Rik, AI265272, NS5ATP1, 3-hydroxyisobutyrate dehydrogenase
External IDs	OMIM: 608475; MGI: 1889802; HomoloGene: 15088; GeneCards: HIBADH; OMA:HIBADH - orthologs
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	2GF2, 2I9P
Enzyme activity
Gene location (Human)
Chr.	Chromosome 7 (human)
End	27,662,883 bp
Gene location (Mouse)
Chr.	Chromosome 6 (mouse)
End	52,617,374 bp
RNA expression pattern
	Top expressed in
	myocardium of left ventricle; ; cardiac muscle tissue of right atrium; ; renal medulla; ; right adrenal cortex; ; human kidney; ; left adrenal gland; ; quadriceps femoris muscle; ; left adrenal cortex; ; vastus lateralis muscle; ; deltoid muscle;
	Top expressed in
	right kidney; ; brown adipose tissue; ; epithelium of stomach; ; proximal tubule; ; interventricular septum; ; lacrimal gland; ; myocardium of ventricle; ; left lobe of liver; ; right ventricle; ; digastric muscle;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	3-hydroxyisobutyrate dehydrogenase activity; oxidoreductase activity; NAD binding; phosphogluconate dehydrogenase (decarboxylating) activity; NADP binding; oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor;
Cellular component	mitochondrial matrix; mitochondrion;
Biological process	valine catabolic process; branched-chain amino acid catabolic process;
	Sources:Amigo / QuickGO
Orthologs
	11112
	58875
	ENSG00000106049
	ENSMUSG00000029776
	P31937
	Q99L13
	NM_152740
	NM_145567
	NP_689953
	NP_663542
	Wikidata
View/Edit Human	View/Edit Mouse

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PMC	articles
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3-hydroxyisobutyrate dehydrogenase
3-hydroxyisobutyrate dehydrogenase
Identifiers
EC no.	1.1.1.31
CAS no.	9028-39-1
Databases
BRENDA	enzyme data
ExPASy	NiceZyme view
KEGG	enzyme entry
MetaCyc	metabolic pathway
Rhea	reactions
PDB structures	RCSB PDB PDBe PDBsum
Gene Ontology	AmiGO / QuickGO
PMC
Search
PMC	articles
PubMed	articles
NCBI	proteins

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-Hydroxyisobutyric acid

+ NAD⁺

H⁺

Reversible left-right reaction arrow with minor forward product(s) to top right and minor reverse substrate(s) from bottom right

H⁺

Methylmalonic acid semialdehyde

+ NADPH

The two substrates of this enzyme are 3-hydroxyisobutyric acid and oxidised nicotinamide adenine dinucleotide (NAD⁺). Its products are methylmalonic acid semialdehyde, reduced NADH, and a proton.^[7]

This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with NAD⁺ or NADP⁺ as acceptor. The systematic name of this enzyme class is 3-hydroxy-2-methylpropanoate:NAD⁺ oxidoreductase. This enzyme participates in 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 PDB: 1WP4, PDB: 2CVZ, PDB: 2GF2, PDB: 2H78, and PDB: 2I9P.

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000106049 – Ensembl, May 2017
1 2 3 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.
↑ Robinson WG, Coon MJ (March 1957). "The purification and properties of beta-hydroxyisobutyric dehydrogenase". The Journal of Biological Chemistry. 225 (1): 511–521. doi:10.1016/S0021-9258(18)64948-8. PMID 13416257.
1 2 "Entrez Gene: HIBADH 3-hydroxyisobutyrate dehydrogenase".
↑ Enzyme 1.1.1.31 at KEGG Pathway Database.

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

This article on a gene on human chromosome 7 is a stub. You can help Wikipedia by adding missing information.

This EC 1.1.1 enzyme-related article is a stub. You can help Wikipedia by adding missing information.

[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000106049 – Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000029776 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid13416257-5] Robinson WG, Coon MJ (March 1957). "The purification and properties of beta-hydroxyisobutyric dehydrogenase". The Journal of Biological Chemistry. 225 (1): 511–521. doi:10.1016/S0021-9258(18)64948-8. PMID 13416257.

[entrez-6] 1 2 "Entrez Gene: HIBADH 3-hydroxyisobutyrate dehydrogenase".

[7] Enzyme 1.1.1.31 at KEGG Pathway Database.

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[7]

v t e Oxidoreductases: alcohol oxidoreductases (EC 1.1)
1.1.1: NAD/NADP acceptor	3-hydroxyacyl-CoA dehydrogenase 3-hydroxybutyryl-CoA dehydrogenase 6-Phosphogluconate dehydrogenase Alcohol dehydrogenase Aldo-keto reductase 1A1 1B1 1B10 1C1 1C3 1C4 7A2 Aldose reductase Beta-Ketoacyl ACP reductase Carbohydrate dehydrogenases Carnitine dehydrogenase D-malate dehydrogenase (decarboxylating) DXP reductoisomerase Glucose-6-phosphate dehydrogenase Glycerol-3-phosphate dehydrogenase HMG-CoA reductase IMP dehydrogenase Isocitrate dehydrogenase Lactate dehydrogenase L-threonine dehydrogenase L-xylulose reductase Malate dehydrogenase Malate dehydrogenase (decarboxylating) Malate dehydrogenase (NADP+) Malate dehydrogenase (oxaloacetate-decarboxylating) Malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) Sorbitol dehydrogenase Hydroxysteroid dehydrogenase: 3β 3β-HSD NSDHL 11β 11β-HSD1 11β-HSD2 17β
1.1.2: cytochrome acceptor	D-lactate dehydrogenase (cytochrome) D-lactate dehydrogenase (cytochrome c-553) Mannitol dehydrogenase (cytochrome)
1.1.3: oxygen acceptor	Alcohol oxidase Glucose oxidase L-gulonolactone oxidase L-lactate oxidase Xanthine oxidase
1.1.4: disulfide as acceptor	Vitamin K epoxide reductase Vitamin-K-epoxide reductase (warfarin-insensitive)
1.1.5: quinone/similar acceptor	Malate dehydrogenase (quinone) Quinoprotein glucose dehydrogenase
1.1.99: other acceptors	Choline dehydrogenase L2HGDH

v t e Enzymes
Activity	Active site Binding site Catalytic triad Oxyanion hole Enzyme promiscuity Diffusion-limited enzyme Cofactor Enzyme catalysis
Regulation	Allosteric regulation Cooperativity Enzyme inhibitor Enzyme activator
Classification	EC number Enzyme superfamily Enzyme family List of enzymes
Kinetics	Enzyme kinetics Eadie–Hofstee diagram Hanes–Woolf plot Lineweaver–Burk plot Michaelis–Menten kinetics
Types	EC1 Oxidoreductases (list) EC2 Transferases (list) EC3 Hydrolases (list) EC4 Lyases (list) EC5 Isomerases (list) EC6 Ligases (list) EC7 Translocases (list)

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3-hydroxyisobutyrate dehydrogenase

Function

Structural studies

References

Further reading

External links