UniProt Accession

 HCDH_HUMAN; Q16836; J3KQ17; O00324; O00397; O00753; Q4W5B4 

Genbank Protein ID

 X96752; AF001902; AF001903; AF001904; AF095703; AC114733; AC118062; BC000306 

Genbank Nucleotide ID

 CAA65528.1; AAB54008.1; AAB54009.1; AAB58153.1; AAD13581.1; AAY41050.1; AAH00306.1 

Protein Name

 Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial 

Protein Synonyms/Alias

 HCDH; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase 

Gene Name

 HADH 

Gene Synonyms/Alias

 HAD; HADHSC; SCHAD 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
154	AGDEFVEKTLSTIAT	ubiquitination	[1]
195	ELFKRLDKFAAEHTI	ubiquitination	[2]
261	ESLVDFSKALGKHPV	ubiquitination	[1]
265	DFSKALGKHPVSCKD	ubiquitination	[1]
271	GKHPVSCKDTPGFIV	ubiquitination	[1]
317	SLKGDASKEDIDTAM	ubiquitination	[1]

Reference

 [1] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [2] Methods for quantification of in vivo changes in protein ubiquitination following proteasome and deubiquitinase inhibition.
 Udeshi ND, Mani DR, Eisenhaure T, Mertins P, Jaffe JD, Clauser KR, Hacohen N, Carr SA.
 Mol Cell Proteomics. 2012 May;11(5):148-59. [PMID: 22505724] 

Functional Description

 Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA. 

Sequence Annotation

 NP_BIND 34 39 NAD.
 BINDING 57 57 NAD.
 BINDING 73 73 Coenzyme A.
 BINDING 80 80 Coenzyme A.
 BINDING 122 122 NAD.
 BINDING 127 127 NAD.
 BINDING 149 149 Coenzyme A.
 BINDING 149 149 NAD.
 BINDING 173 173 NAD.
 BINDING 305 305 NAD.
 MOD_RES 185 185 N6-acetyllysine.
 MOD_RES 202 202 N6-acetyllysine.
 MOD_RES 241 241 N6-acetyllysine.
 MOD_RES 312 312 N6-acetyllysine.  

Keyword

 3D-structure; Acetylation; Alternative splicing; Complete proteome; Disease mutation; Fatty acid metabolism; Lipid metabolism; Mitochondrion; NAD; Oxidoreductase; Polymorphism; Reference proteome; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 314 AA 

Protein Sequence

Gene Ontology

 GO:0005759; C:mitochondrial matrix; TAS:Reactome.
 GO:0005634; C:nucleus; IDA:HPA.
 GO:0003857; F:3-hydroxyacyl-CoA dehydrogenase activity; TAS:Reactome.
 GO:0070403; F:NAD+ binding; IEA:InterPro.
 GO:0006635; P:fatty acid beta-oxidation; TAS:Reactome. 

Interpro

 IPR022694; 3-OHacyl-CoA_DH.
 IPR006180; 3-OHacyl-CoA_DH_CS.
 IPR006176; 3-OHacyl-CoA_DH_NAD-bd.
 IPR006108; 3HC_DH_C.
 IPR008927; 6-PGluconate_DH_C-like.
 IPR013328; DH_multihelical.
 IPR016040; NAD(P)-bd_dom. 

Pfam

 PF00725; 3HCDH
 PF02737; 3HCDH_N 

SMART

  

PROSITE

 PS00067; 3HCDH 

PRINTS