LipidDB ID

LipidDB-9606-00037

Entry Name

UniProt Accession

P54868; B7Z8R3; D3Y5K6; Q5SZU2; Q6IBF4;

Theoretical PI

8.4

Molecular Weight

56635.44

Genbank Protein ID

CAA58593.1; AAB72036.1; AAB72036.1; AAB72036.1; AAB72036.1; AAB72036.1; AAB72036.1; AAB72036.1; AAB72036.1; AAB72036.1; CAG33131.1; BAH14049.1; CAI22408.1; EAW56709.1; AAH44217.1; AAA92673.1; AAA92674.1; ADD21696.1;

Genbank Nucleotide ID

X83618; U81859; U81851; U81852; U81853; U81854; U81855; U81856; U81857; U81858; CR456850; AK303777; AL589734; CH471122; BC044217; U12788; U12789; GU433940;

Protein Name

Hydroxymethylglutaryl-CoA synthase, mitochondrial

Protein Synonyms/Alias

HMG-CoA synthase; 2.3.3.10; 3-hydroxy-3-methylglutaryl coenzyme A synthase;

Gene Name

HMGCS2

Gene Synonyms/Alias

Created Date

01-OCT-1996

Organism

Homo sapiens (Human)

NCBI Taxa ID

9606

Reference

[1] Kostiuk MA, Keller BO, Berthiaume LG. Palmitoylation of ketogenic enzymeHMGCS2 enhances its interaction with PPARalpha and transcription at the Hmgcs2PPRE. FASEB J. 2010 Jun;24(6):1914-24. doi: 10.1096/fj.09-149765. Epub 2010 Feb2.[PMID:20124434]

Functional Description

This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.

Sequence Annotation

Active site: 132 132 Proton donor/acceptor.
Active site: 166 166 Acyl-thioester intermediate.
Active site: 301 301 Proton donor/acceptor.
Binding site: 80 80 Substrate.
Binding site: 204 204 Substrate.
Binding site: 258 258 Substrate.
Binding site: 380 380 Substrate.
Modified residue: 52 52 N6-succinyllysine.
Modified residue: 83 83 N6-acetyllysine; alternate.
Modified residue: 83 83 N6-succinyllysine; alternate.
Modified residue: 221 221 N6-succinyllysine.
Modified residue: 243 243 N6-acetyllysine.
Modified residue: 256 256 N6-acetyllysine; alternate.
Modified residue: 256 256 N6-succinyllysine; alternate.
Modified residue: 306 306 N6-acetyllysine.
Modified residue: 310 310 N6-acetyllysine; alternate.
Modified residue: 310 310 N6-succinyllysine; alternate.
Modified residue: 333 333 N6-succinyllysine.
Modified residue: 342 342 N6-acetyllysine; alternate.
Modified residue: 342 342 N6-succinyllysine; alternate.
Modified residue: 350 350 N6-acetyllysine; alternate.
Modified residue: 350 350 N6-succinyllysine; alternate.
Modified residue: 354 354 N6-acetyllysine; alternate.
Modified residue: 354 354 N6-succinyllysine; alternate.
Modified residue: 358 358 N6-acetyllysine; alternate.
Modified residue: 358 358 N6-succinyllysine; alternate.
Modified residue: 437 437 N6-acetyllysine.
Modified residue: 447 447 N6-acetyllysine; alternate.
Modified residue: 447 447 N6-succinyllysine; alternate.
Modified residue: 473 473 N6-acetyllysine; alternate.
Modified residue: 473 473 N6-succinyllysine; alternate.

Protein Length

508 AA.

Protein Sequence
(Canonical)

FASTA
(Canonical)

Gene Ontology

GO:0005743; C:mitochondrial inner membrane; IEA:Ensembl
GO:0005759; C:mitochondrial matrix; TAS:Reactome
GO:0005739; C:mitochondrion; TAS:ProtInc
GO:0004421; F:hydroxymethylglutaryl-CoA synthase activity; IEA:UniProtKB-EC
GO:0046950; P:cellular ketone body metabolic process; TAS:Reactome
GO:0044255; P:cellular lipid metabolic process; TAS:Reactome
GO:0006695; P:cholesterol biosynthetic process; IEA:UniProtKB-KW
GO:0008299; P:isoprenoid biosynthetic process; IEA:InterPro
GO:0046951; P:ketone body biosynthetic process; TAS:Reactome
GO:0044281; P:small molecule metabolic process; TAS:Reactome

Interpro

InterPro; IPR000590; HMG_CoA_synt_AS
InterPro; IPR013746; HMG_CoA_synt_C_dom
InterPro; IPR013528; HMG_CoA_synth_N
InterPro; IPR010122; HMG_CoA_synthase_euk
InterPro; IPR016039; Thiolase-like
InterPro; IPR016038; Thiolase-like_subgr

Pfam

Pfam; PF08540; HMG_CoA_synt_C;
Pfam; PF01154; HMG_CoA_synt_N;

SMART

PROSITE

PROSITE; PS01226; HMG_COA_SYNTHASE;

PRINTS