UniProt Accession

 3HIDH_HUMAN; P31937; Q546Z2; Q9UDN3 

Genbank Protein ID

 AF529362; AB050000; AK316605; AC007130; AC005091; CH236948; CH471073; BC032324 

Genbank Nucleotide ID

 AAQ09596.1; BAF42045.1; BAG38192.1; EAL24214.1; EAW93897.1; AAH32324.1 

Protein Name

 3-hydroxyisobutyrate dehydrogenase, mitochondrial 

Protein Synonyms/Alias

 HIBADH 

Gene Name

 HIBADH 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
95	SPADVAEKADRIITM	ubiquitination	[1]
141	TIDPAVSKELAKEVE	ubiquitination	[1]
297	QDSATSTKSPILLGS	ubiquitination	[1]
321	CAKGYSKKDFSSVFQ	ubiquitination	[2, 3]

Reference

 [1] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661]
 [2] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [3] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789] 

Functional Description

  

Sequence Annotation

 NP_BIND 40 68 NAD.
 NP_BIND 103 104 NAD.
 ACT_SITE 209 209 By similarity.
 BINDING 108 108 NAD.
 BINDING 134 134 NAD.
 BINDING 284 284 NAD.  

Keyword

 3D-structure; Branched-chain amino acid catabolism; Complete proteome; Direct protein sequencing; Mitochondrion; NAD; Oxidoreductase; Reference proteome; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 336 AA 

Protein Sequence

Gene Ontology

 GO:0005759; C:mitochondrial matrix; TAS:Reactome.
 GO:0008442; F:3-hydroxyisobutyrate dehydrogenase activity; IDA:UniProtKB.
 GO:0051287; F:NAD binding; IEA:InterPro.
 GO:0004616; F:phosphogluconate dehydrogenase (decarboxylating) activity; IEA:InterPro.
 GO:0034641; P:cellular nitrogen compound metabolic process; TAS:Reactome.
 GO:0006098; P:pentose-phosphate shunt; IEA:InterPro.
 GO:0006574; P:valine catabolic process; IDA:UniProtKB. 

Interpro

 IPR002204; 3-OH-isobutyrate_DH-rel_CS.
 IPR008927; 6-PGluconate_DH_C-like.
 IPR006115; 6PGDH_NADP-bd.
 IPR013328; DH_multihelical.
 IPR015815; HIBADH-type.
 IPR011548; IsoBut3OH_DH.
 IPR016040; NAD(P)-bd_dom. 

Pfam

 PF03446; NAD_binding_2 

SMART

  

PROSITE

 PS00895; 3_HYDROXYISOBUT_DH 

PRINTS