3-羟基异丁酸脱氢酶

3-羟基异丁酸脱氢酶（英语：3-hydroxyisobutyrate dehydrogenase，EC 1.1.1.31（页面存档备份，存于互联网档案馆））是一种以NAD⁺或NADP⁺为受体、作用于供体CH-OH基团上的氧化还原酶。这种酶能催化以下酶促反应：

3-羟基异丁酸脱氢酶
PDB rendering based on 2gf2.
有效结构 PDB 直系同源检索：PDBe, RCSB PDB查询代码列表 2GF2, 2I9P
标识
代号	HIBADH; NS5ATP1
扩展标识	遗传学：608475 鼠基因：1889802 同源基因：15088 GeneCards: HIBADH Gene
EC编号	1.1.1.31
基因本体论描述 分子功能 · phosphogluconate dehydrogenase (decarboxylating) activity · 3-hydroxyisobutyrate dehydrogenase activity · NAD binding 细胞成分 · mitochondrion · mitochondrial matrix 生物过程 · pentose-phosphate shunt · valine catabolic process · branched-chain amino acid catabolic process · cellular nitrogen compound metabolic process · small molecule metabolic process Sources: Amigo / QuickGO
RNA表达模式
更多表达数据
直系同源体
物种	人类	小鼠
Entrez	11112	58875
Ensembl	ENSG00000106049	ENSMUSG00000029776
UniProt	P31937	Q99L13
mRNA序列	NM_152740	NM_145567
蛋白序列	NP_689953	NP_663542
基因位置	Chr 7: 27.57 – 27.7 Mb	Chr 6: 52.55 – 52.64 Mb
PubMed查询	[1]	[2]

3-羟基异丁酸脱氢酶
3-羟基异丁酸脱氢酶结构（PDB 2gf2）
命名 系统命名 3-hydroxy-2-methylpropanoate:NAD+ oxidoreductase 缩写 HIBADH 别名 β-hydroxyisobutyrate dehydrogenase
识别码
EC编号	1.1.1.31
CAS号	9028-39-1
数据库
IntEnz	IntEnz浏览
BRENDA	BRENDA入口
ExPASy	NiceZyme浏览
KEGG	KEGG入口
MetaCyc	代谢路径
PRIAM	概述
PDB	RCSB PDB PDBj PDBe PDBsum
基因本体	AmiGO / EGO
搜索 PMC 相关文献 PubMed 相关文献

3-羟基-2-甲基丙酸 + NAD⁺ $\rightleftharpoons$ 2-甲基-3-氧代丙酸 + NADH + H⁺

3-羟基异丁酸脱氢酶主要参与缬氨酸、亮氨酸和异亮氨酸的降解过程。

参考文献

• Gerhard DS; Wagner L; Feingold EA; et al. The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).. Genome Res. 2004, 14 (10B): 2121–7. PMC 528928 . PMID 15489334. doi:10.1101/gr.2596504. 
• Hillier LW; Fulton RS; Fulton LA; et al. The DNA sequence of human chromosome 7.. Nature. 2003, 424 (6945): 157–64. PMID 12853948. doi:10.1038/nature01782. 
• Scherer SW; Cheung J; MacDonald JR; et al. Human chromosome 7: DNA sequence and biology.. Science. 2003, 300 (5620): 767–72. PMC 2882961 . PMID 12690205. doi:10.1126/science.1083423. 
• Strausberg RL; Feingold EA; Grouse LH; et al. Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.. Proc. Natl. Acad. Sci. U.S.A. 2003, 99 (26): 16899–903. PMC 139241 . PMID 12477932. doi:10.1073/pnas.242603899. 
• Toward a complete human genome sequence.. Genome Res. 1999, 8 (11): 1097–108. PMID 9847074. doi:10.1101/gr.8.11.1097. 
• Hughes GJ; Frutiger S; Paquet N; et al. Human liver protein map: update 1993.. Electrophoresis. 1994, 14 (11): 1216–22. PMID 8313870. doi:10.1002/elps.11501401181. 
• Rougraff PM; Paxton R; Kuntz MJ; et al. Purification and characterization of 3-hydroxyisobutyrate dehydrogenase from rabbit liver.. J. Biol. Chem. 1988, 263 (1): 327–31. PMID 3335502. 
• Rougraff PM; Zhang B; Kuntz MJ,; et al. 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. 1989, 264 (10): 5899–903. PMID 2647728.