Mus musculus Gene: Hmgcl
Summary
InnateDB Gene IDBG-197422.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol Hmgcl
Gene Name 3-hydroxy-3-methylglutaryl-Coenzyme A lyase
Synonyms AW476067; HL
Species Mus musculus
Ensembl Gene ENSMUSG00000028672
Encoded Proteins
3-hydroxy-3-methylglutaryl-Coenzyme A lyase
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary This gene does not have any Entrez summary - the following is the summary from its human ortholog ENSG00000117305:
The protein encoded by this gene belongs to the HMG-CoA lyase family. It is a mitochondrial enzyme that catalyzes the final step of leucine degradation and plays a key role in ketone body formation. Mutations in this gene are associated with HMG-CoA lyase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
Gene Information
Type Protein coding
Genomic Location Chromosome 4:135946448-135962617
Strand Forward strand
Band D3
Transcripts
ENSMUST00000030432 ENSMUSP00000030432
ENSMUST00000134135
ENSMUST00000151879
ENSMUST00000148613
ENSMUST00000135644
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 4 experimentally validated interaction(s) in this database.
They are also associated with 8 interaction(s) predicted by orthology.
Experimentally validated
Total 4 [view]
Protein-Protein 3 [view]
Protein-DNA 1 [view]
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 8 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0000062 fatty-acyl-CoA binding
GO:0000287 magnesium ion binding
GO:0003824 catalytic activity
GO:0004419 hydroxymethylglutaryl-CoA lyase activity
GO:0005102 receptor binding
GO:0030145 manganese ion binding
GO:0031406 carboxylic acid binding
GO:0042803 protein homodimerization activity
GO:0046872 metal ion binding
Biological Process
GO:0001889 liver development
GO:0006637 acyl-CoA metabolic process
GO:0007005 mitochondrion organization
GO:0007584 response to nutrient
GO:0042594 response to starvation
GO:0046951 ketone body biosynthetic process
GO:0051262 protein tetramerization
GO:0070542 response to fatty acid
Cellular Component
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0005759 mitochondrial matrix
GO:0005777 peroxisome
Orthologs
Species
Homo sapiens
Bos taurus
Gene ID
Gene Order
Not yet available
Pathways
NETPATH
REACTOME
Fatty acid, triacylglycerol, and ketone body metabolism pathway
Metabolism pathway
Metabolism of lipids and lipoproteins pathway
Synthesis of Ketone Bodies pathway
Ketone body metabolism pathway
KEGG
Valine, leucine and isoleucine degradation pathway
Synthesis and degradation of ketone bodies pathway
Butanoate metabolism pathway
Peroxisome pathway
INOH
PID NCI
Pathway Predictions based on Human Orthology Data
NETPATH
REACTOME
Synthesis of Ketone Bodies pathway
Fatty acid, triacylglycerol, and ketone body metabolism pathway
Metabolism of lipids and lipoproteins pathway
Ketone body metabolism pathway
Metabolism pathway
Synthesis of Ketone Bodies pathway
Ketone body metabolism pathway
Metabolism of lipids and lipoproteins pathway
Metabolism pathway
Fatty acid, triacylglycerol, and ketone body metabolism pathway
KEGG
Valine, leucine and isoleucine degradation pathway
Butanoate metabolism pathway
Synthesis and degradation of ketone bodies pathway
Peroxisome pathway
INOH
Butanoate metabolism pathway
Pyruvate metabolism pathway
Valine Leucine Isoleucine degradation pathway
PID NCI
Cross-References
SwissProt
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Mm.482102 Mm.486632
RefSeq NM_008254 XM_006538563
OMIM
CCDS CCDS18795
HPRD
IMGT
MGI ID
MGI Symbol
EMBL
GenPept
RNA Seq Atlas