Gene Information
Gene NameHMGCL
OrganismHomo sapiens (Human)
Gene Length325
Protein NamesHydroxymethylglutaryl-CoA lyase, mitochondrial (HL) (HMG-CoA lyase) (EC 4.1.3.4) (3-hydroxy-3-methylglutarate-CoA lyase)
Target Namehydroxymethylglutaryl-coa lyase, mitochondrial
Target TypeNA
Gene Age1488 ( ENSG00000117305 )
Evolutionary StageEuk+Bac
AlphaFoldDBP35914
Gene CardHMGCL
Uniprot IDP35914
PfamPF00682
In ASDno
Allosteric Predictionyes (0.237156378)
Ortholog in AnimalsAlpaca_ HMGCL ( ortholog_one2one )
Dog_ HMGCL ( ortholog_one2one )
GuineaPig_ HMGCL ( ortholog_one2one )
Macaque_ HMGCL ( ortholog_one2one )
Mouse_ Hmgcl ( ortholog_one2one )
Pig_ HMGCL ( ortholog_one2one )
Rat_ Hmgcl ( ortholog_one2one )
OhnologsHMGCLL1
ParalogsHMGCLL1
TissueNA
Tissue SpecificityHighest expression in liver. Expressed in pancreas, kidney, intestine, testis, fibroblasts and lymphoblasts. Very low expression in brain and skeletal muscle. The relative expression of isoform 2 (at mRNA level) is highest in heart (30%), skeletal muscle (22%), and brain (14%). {ECO:0000269|PubMed:21952825}.
Pharmacological Animal ModelsNA
Gene Ontology
(biological process)
Gene Ontology
(cellular component)
Gene Ontology
(molecular function)
PDB 2CW6  (X-ray  2.10A  3MP3  (X-ray  2.40A  3MP4  (X-ray  2.20A  3MP5  (X-ray  2.25A 
DBSNP rs121964997  rs1467902610  rs121964996  rs1357942068  rs199587895  rs765475941  rs760106433  rs1553131940  rs727503963  rs121964998 
Subcellular location [CC]Mitochondrion matrix {ECO:0000250|UniProtKB:P38060}. Peroxisome {ECO:0000250|UniProtKB:P38060}. Note=Unprocessed form is peroxisomal. {ECO:0000250|UniProtKB:P38060}.
Mouse PG classificationLPG
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