UniProt Accession

 HCD2_HUMAN; Q99714; Q5H927; Q8TCV9; Q96HD5 

Genbank Protein ID

 U96132; U73514; AF069134; AF035555; AF037438; Z97054; CH471154; BC000372; BC008708; AY092415 

Genbank Nucleotide ID

 AAC51812.1; AAB68958.1; AAC39900.1; AAC15902.1; AAC16419.1; CAI42652.1; EAW93158.1; AAH00372.1; AAH08708.1; AAM18189.1 

Protein Name

 3-hydroxyacyl-CoA dehydrogenase type-2 

Protein Synonyms/Alias

 17-beta-hydroxysteroid dehydrogenase 10; 17-beta-HSD 10; 3-hydroxy-2-methylbutyryl-CoA dehydrogenase; 3-hydroxyacyl-CoA dehydrogenase type II; Endoplasmic reticulum-associated amyloid beta-peptide-binding protein; Mitochondrial ribonuclease P protein 2; Mitochondrial RNase P protein 2; Short-chain type dehydrogenase/reductase XH98G2; Type II HADH 

Gene Name

 HSD17B10 

Gene Synonyms/Alias

 ERAB; HADH2; MRPP2; SCHAD; XH98G2 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
52	SGGEAQAKKLGNNCV	ubiquitination	[1, 2]
53	GGEAQAKKLGNNCVF	ubiquitination	[2]
79	QTALALAKGKFGRVD	ubiquitination	[1]
99	AGIAVASKTYNLKKG	ubiquitination	[2, 3]
105	SKTYNLKKGQTHTLE	ubiquitination	[2]

Reference

 [1] Systematic and quantitative assessment of the ubiquitin-modified proteome.
 Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
 Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983]
 [2] 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]
 [3] 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] 

Functional Description

 Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD). 

Sequence Annotation

 NP_BIND 12 41 NAD.
 ACT_SITE 168 168 Proton acceptor.
 BINDING 155 155 Substrate.
 BINDING 172 172 NAD.
 MOD_RES 2 2 N-acetylalanine (By similarity).  

Keyword

 3D-structure; Acetylation; Alternative splicing; Chromosomal rearrangement; Complete proteome; Disease mutation; Mental retardation; Mitochondrion; NAD; Oxidoreductase; Reference proteome; tRNA processing. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 261 AA 

Protein Sequence

Gene Ontology

 GO:0005783; C:endoplasmic reticulum; IEA:Compara.
 GO:0005743; C:mitochondrial inner membrane; IEA:Compara.
 GO:0005759; C:mitochondrial matrix; TAS:Reactome.
 GO:0005886; C:plasma membrane; TAS:ProtInc.
 GO:0047015; F:3-hydroxy-2-methylbutyryl-CoA dehydrogenase activity; IEA:EC.
 GO:0003857; F:3-hydroxyacyl-CoA dehydrogenase activity; EXP:Reactome.
 GO:0008709; F:cholate 7-alpha-dehydrogenase activity; TAS:ProtInc.
 GO:0009083; P:branched-chain amino acid catabolic process; TAS:Reactome.
 GO:0034641; P:cellular nitrogen compound metabolic process; TAS:Reactome.
 GO:0006629; P:lipid metabolic process; TAS:ProtInc.
 GO:0008033; P:tRNA processing; IEA:UniProtKB-KW. 

Interpro

 IPR002198; DH_sc/Rdtase_SDR.
 IPR002347; Glc/ribitol_DH.
 IPR016040; NAD(P)-bd_dom.
 IPR020904; Sc_DH/Rdtase_CS. 

Pfam

 PF00106; adh_short 

SMART

  

PROSITE

 PS00061; ADH_SHORT 

PRINTS

 PR00081; GDHRDH.
 PR00080; SDRFAMILY.