MGP Database

MGP001465

Ontology/Pathway Information

Entrez Gene ID3155
Gene Name3-hydroxymethyl-3-methylglutaryl-CoA lyase
Gene Symbol HMGCL
SpeciesHomo sapiens
Gene Ontology (GO Annotations)
GO IDSourceTypeDescription
GO:0005743 IEA:EnsemblCmitochondrial inner membrane
GO:0005759 NAS:UniProtKBCmitochondrial matrix
GO:0005739 IDA:UniProtKBCmitochondrion
GO:0005777 IDA:UniProtKBCperoxisome
GO:0031406 IEA:EnsemblFcarboxylic acid binding
GO:0000062 IEA:EnsemblFfatty-acyl-CoA binding
GO:0004419 IDA:UniProtKBFhydroxymethylglutaryl-CoA lyase activity
GO:0000287 IDA:UniProtKBFmagnesium ion binding
GO:0030145 IDA:UniProtKBFmanganese ion binding
GO:0046872 IDA:UniProtKBFmetal ion binding
GO:0042803 IDA:UniProtKBFprotein homodimerization activity
GO:0005102 IPI:UniProtKBFreceptor binding
GO:0006637 IEA:EnsemblPacyl-CoA metabolic process
GO:0046950 TAS:ReactomePcellular ketone body metabolic process
GO:0044255 TAS:ReactomePcellular lipid metabolic process
GO:0046951 IDA:UniProtKBPketone body biosynthetic process
GO:0006552 NAS:UniProtKBPleucine catabolic process
GO:0001889 IEA:EnsemblPliver development
GO:0007005 IEA:EnsemblPmitochondrion organization
GO:0051262 IDA:UniProtKBPprotein tetramerization
GO:0070542 IEA:EnsemblPresponse to fatty acid
GO:0007584 IEA:EnsemblPresponse to nutrient
GO:0042594 IEA:EnsemblPresponse to starvation
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_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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