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0HMCS2_HUMAN*   SwissProt (?) | Description Local Annotation Link Reference
General Information
NameHMGCS2
DescriptionHydroxymethylglutaryl-coa synthase, mitochondrial precursor (ec 2.3.3.10) (hmg-coa synthase) (3-hydroxy-3-methylglutaryl coenzyme a synthase).
SpeciesHomo sapiens (NCBI taxonomy ID: 9606)
GO0005739 mitochondrion (TAS)
Domain Architecture (Details)
InterPro domains unassigned to SynO:
This domain is found at the C-terminus of hydroxymethylglutaryl-coenzyme A synthase (HMG-CoA synthase) that catalyzes the condensation ofacetyl-CoA with acetoacetyl-CoA to produce HMG-CoA and CoA . A cysteine is known to act as the catalyticnucleophile in the first step of the reaction.he acetylation of the enzyme by acetyl-CoA. In vertebrates there aretwo isozymes located in different subcellular compartments. cytosolic form which is the starting point of themevalonate pathway which leads to cholesterol and other sterolic and isoprenoid compounds.nd a mitochondrial formresponsible for ketone body biosynthesis. HMG-CoA is also found in other eukaryotes such as insects.lants and fungi.
  IPR013746:Hydroxymethylglutaryl-coenzyme A synthase C-terminal
Hydroxymethylglutaryl-coenzyme A synthase (HMG-CoA synthase) catalyzes the condensation ofacetyl-CoA with acetoacetyl-CoA to produce HMG-CoA and CoA . A cysteine is known to act as the catalyticnucleophile in the first step of the reaction.he acetylation of the enzyme by acetyl-CoA. In vertebrates there aretwo isozymes located in different subcellular compartments. cytosolic form which is the starting point of themevalonate pathway which leads to cholesterol and other sterolic and isoprenoid compounds.nd a mitochondrial formresponsible for ketone body biosynthesis. HMG-CoA is also found in other eukaryotes such as insects.lants and fungi.
  IPR013528:Hydroxymethylglutaryl-coenzyme A synthase, N-terminal
Hydroxymethylglutaryl-coenzyme A synthase (HMG-CoA synthase) catalyzes the condensation ofacetyl-CoA with acetoacetyl-CoA to produce HMG-CoA and CoA . A cysteine is known to act as the catalyticnucleophile in the first step of the reaction.he acetylation of the enzyme by acetyl-CoA. In vertebrates there aretwo isozymes located in different subcellular compartments. cytosolic form which is the starting point of themevalonate pathway which leads to cholesterol and other sterolic and isoprenoid compounds.nd a mitochondrial formresponsible for ketone body biosynthesis. HMG-CoA is also found in other eukaryotes such as insects.lants and fungi.
  IPR008260:Hydroxymethylglutaryl-coenzyme A synthase
Hydroxymethylglutaryl(HMG)-CoA synthase is the first step of isopentenyl pyrophosphate (IPP) biosynthesis via the mevalonate pathway. This pathway is found mainly in eukaryotes.ut also in archaea and some bacteria. This entry is specific for eukaryotes.
  IPR010122:Hydroxymethylglutaryl-CoA synthase
IPR013746:HMG_CoA_synt_C 
Evalue:-203.619781494141 
Location:224-506IPR013528:HMG_CoA_synt_N 
Evalue:-128.119186401367 
Location:50-223
SequencesProtein: HMCS2_HUMAN (508 aa)
mRNA: NM_005518
Local Annotation
Synapse Ontology
mitochondria are frequently observed in the vicinity of the synaptic vesicle clusters, in agreement with the ATP requirement of several steps of the vesicle cycle.
sdb:0118 mitochondria  (Evidence:keywords)
KO assignmentK01641
  Level 3 annotation:
    hydroxymethylglutaryl-CoA synthase
  Level 2 annotation:
    Butanoate metabolism
    Synthesis and degradation of ketone bodies
    Valine
     leucine and isoleucine degradation
Loci Structure (Details)Loci index, Chromosomal location, Length, Possible relational loci clusterExon1: 154 residues, 120092527-120092987Exon2: 39 residues, 120094942-120095054Exon3: 44 residues, 120096694-120096820Exon4: 37 residues, 120097425-120097532Exon5: 59 residues, 120099572-120099743Exon6: 57 residues, 120101418-120101584Exon7: 57 residues, 120103263-120103428Exon8: 44 residues, 120104009-120104135Exon9: 153 residues, 120108317-120108772Exon10: 53 residues, 120112886-120113041Exon11: 2 residues, -Jump to HMCS2_HUMAN  
Tune and view alternative isoforms
Loci Cluster (Details)Loci: 2544 120056032-120088359 ~-32K 2308(PHGDH)(+)Loci: 3825 120092527-120113041 ~-21K 2310(HMGCS2)(-)Loci: 3826 120255700-120413799 ~-158K 2313(NOTCH2)(-)Loci: 2543 119759295-119767172 ~-8K 2303(HSD3B2)(+)Link out to UCSC