MGP Database

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MGP001468

Ontology/Pathway Information

Entrez Gene ID3158
Gene Name3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial)
Gene Symbol HMGCS2
SpeciesHomo sapiens
Gene Ontology (GO Annotations)
GO IDSourceTypeDescription
GO:0005743 IEA:EnsemblCmitochondrial inner membrane
GO:0005759 TAS:ReactomeCmitochondrial matrix
GO:0005739 TAS:ProtIncCmitochondrion
GO:0004421 IEA:UniProtKB-ECFhydroxymethylglutaryl-CoA synthase activity
GO:0046950 TAS:ReactomePcellular ketone body metabolic process
GO:0044255 TAS:ReactomePcellular lipid metabolic process
GO:0006695 IEA:UniProtKB-KWPcholesterol biosynthetic process
GO:0008299 IEA:InterProPisoprenoid biosynthetic process
GO:0046951 TAS:ReactomePketone body biosynthetic process
GO:0044281 TAS:ReactomePsmall molecule metabolic process
REACTOME Pathway Links
REACTOME Pathway IDDescription
REACT_22279Fatty acid, triacylglycerol, and ketone body metabolism
REACT_1861Ketone body metabolism
REACT_111217Metabolism
REACT_22258Metabolism of lipids and lipoproteins
REACT_116145PPARA activates gene expression
REACT_19241Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha)
REACT_1464Synthesis of Ketone Bodies
SMP Pathway Links
SMP IDDescription
SMP001372-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency
SMP001383-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
SMP005213-hydroxyisobutyric acid dehydrogenase deficiency
SMP005223-hydroxyisobutyric aciduria
SMP002373-Methylcrotonyl Coa Carboxylase Deficiency Type I
SMP001393-Methylglutaconic Aciduria Type I
SMP001403-Methylglutaconic Aciduria Type III
SMP001413-Methylglutaconic Aciduria Type IV
SMP00173Beta-Ketothiolase Deficiency
SMP00073Butyrate Metabolism
SMP00523Isobutyryl-coa dehydrogenase deficiency
SMP00524Isovaleric acidemia
SMP00238Isovaleric Aciduria
SMP00071Ketone Body Metabolism
SMP00199Maple Syrup Urine Disease
SMP00384Methylmalonate Semialdehyde Dehydrogenase Deficiency
SMP00200Methylmalonic Aciduria
SMP00236Propionic Acidemia
SMP00569Succinyl CoA: 3-ketoacid CoA transferase deficiency
SMP00032Valine, Leucine and Isoleucine Degradation
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