Gene/Proteome Database (LMPD)

LMPD ID
LMP012745
Gene ID
79238
Species
Rattus norvegicus (Rat)
Gene Name
3-hydroxymethyl-3-methylglutaryl-CoA lyase
Gene Symbol
Hmgcl
Alternate Names
hydroxymethylglutaryl-CoA lyase, mitochondrial; HL; HMG-CoA lyase; 3-hydroxy-3-methylglutarate-CoA lyase; 3-hydroxy-3-methylglutaryl-Coenzyme A lyase; 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase;
Chromosome
5
Map Location
5q36
EC Number
4.1.3.4
Summary
an enzyme of the ketogenic 3-hydroxy-3-methylglutaryl-CoA cycle [RGD, Feb 2006]
Orthologs
View orthologs and multiple alignments for Hmgcl

Proteins

hydroxymethylglutaryl-CoA lyase, mitochondrial precursor
Refseq ID NP_077362
Protein GI 13242293
UniProt ID P97519
mRNA ID NM_024386
Length 325
MATVRKAFPQRLVGLASLRAASTSSMGTLPKRVKIVEVGPRDGLQNEKSIVPTPVKIKLIDMLSEAGLPVIEATSFVSPKWVPQMADHSDVLKGIQKFPGINYPVLTPNMKGFEEAVAAGAKEVSIFGAASELFTRKNVNCSIEESFQRFDGVMQAARAASISVRGYVSCALGCPYEGKVSPAKVAEVAKKLYSMGCYEISLGDTIGVGTPGLMKDMLTAVLHEVPVAALAVHCHDTYGQALANTLVALQMGVSVVDSSVAGLGGCPYAKGASGNLATEDLVYMLTGLGIHTGVNLQKLLEAGDFICQALNRKTSSKVAQATCKL
transit_peptide: 1..27 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (P97519.1) calculated_mol_wt: 2807 peptide sequence: MATVRKAFPQRLVGLASLRAASTSSMG mat_peptide: 28..325 product: Hydroxymethylglutaryl-CoA lyase, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P97519.1) calculated_mol_wt: 31403 peptide sequence: TLPKRVKIVEVGPRDGLQNEKSIVPTPVKIKLIDMLSEAGLPVIEATSFVSPKWVPQMADHSDVLKGIQKFPGINYPVLTPNMKGFEEAVAAGAKEVSIFGAASELFTRKNVNCSIEESFQRFDGVMQAARAASISVRGYVSCALGCPYEGKVSPAKVAEVAKKLYSMGCYEISLGDTIGVGTPGLMKDMLTAVLHEVPVAALAVHCHDTYGQALANTLVALQMGVSVVDSSVAGLGGCPYAKGASGNLATEDLVYMLTGLGIHTGVNLQKLLEAGDFICQALNRKTSSKVAQATCKL

Gene Information

Entrez Gene ID
79238
Gene Name
3-hydroxymethyl-3-methylglutaryl-CoA lyase
Gene Symbol
Hmgcl
Species
Rattus norvegicus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005739 IDA:RGD C mitochondrion
GO:0005777 IEA:UniProtKB-KW C peroxisome
GO:0031406 IPI:RGD F carboxylic acid binding
GO:0000062 IPI:RGD F fatty-acyl-CoA binding
GO:0004419 ISS:UniProtKB F hydroxymethylglutaryl-CoA lyase activity
GO:0000287 IEA:Ensembl F magnesium ion binding
GO:0030145 IEA:Ensembl F manganese ion binding
GO:0046872 ISS:UniProtKB F metal ion binding
GO:0006637 IDA:RGD P acyl-CoA metabolic process
GO:0046951 IDA:RGD P ketone body biosynthetic process
GO:0001889 IEP:RGD P liver development
GO:0007005 IEA:Ensembl P mitochondrion organization
GO:0051262 IEA:Ensembl P protein tetramerization
GO:0070542 IEP:RGD P response to fatty acid
GO:0007584 IEP:RGD P response to nutrient
GO:0042594 IEP:RGD P response to starvation

KEGG Pathway Links

KEGG Pathway ID Description
ko00650 Butanoate metabolism
rno00650 Butanoate metabolism
M00036 Leucine degradation, leucine => acetoacetate + acetyl-CoA
rno01100 Metabolic pathways
ko04146 Peroxisome
rno04146 Peroxisome
ko00072 Synthesis and degradation of ketone bodies
rno00072 Synthesis and degradation of ketone bodies
ko00280 Valine, leucine and isoleucine degradation
rno00280 Valine, leucine and isoleucine degradation

REACTOME Pathway Links

REACTOME Pathway ID Description
5953288 Fatty acid, triacylglycerol, and ketone body metabolism
5953366 Ketone body metabolism
5953250 Metabolism
5953289 Metabolism of lipids and lipoproteins
5953365 Synthesis of Ketone Bodies

Domain Information

InterPro Annotations

Accession Description
IPR013785 Aldolase-type TIM barrel
IPR000138 Hydroxymethylglutaryl-CoA lyase, active site
IPR000891 Pyruvate carboxyltransferase

UniProt Annotations

Entry Information

Gene Name
3-hydroxymethyl-3-methylglutaryl-CoA lyase
Protein Entry
HMGCL_RAT
UniProt ID
P97519
Species
Rat

Comments

Comment Type Description
Catalytic Activity (S)-3-hydroxy-3-methylglutaryl-CoA = acetyl- CoA + acetoacetate
Function Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Ketone bodies (beta- hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism (By similarity)
Pathway Metabolic intermediate metabolism; (S)-3-hydroxy-3- methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3- methylglutaryl-CoA: step 1/1.
Similarity Belongs to the HMG-CoA lyase family
Subcellular Location Mitochondrion matrix . Peroxisome . Note=Unprocessed form is peroxisomal
Subunit Homodimer; disulfide-linked. Can also form homotetramers
Tissue Specificity In suckling rat, highest levels in liver and in intestine. Lower levels in heart, kidney and cerebellum. Weak expression in brain cortex, medulla and midbrain. Levels decrease slightly during weaning

Identical and Related Proteins

Unique RefSeq proteins for LMP012745 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
13242293 RefSeq NP_077362 325 hydroxymethylglutaryl-CoA lyase, mitochondrial precursor

Identical Sequences to LMP012745 proteins

Reference Database Accession Length Protein Name
GI:13242293 EMBL CAA71148.1 325 3-hydroxy-3-methylglutaryl CoA lyase [Rattus norvegicus]
GI:13242293 GenBank AAH61797.1 325 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase [Rattus norvegicus]
GI:13242293 GenBank EDL80779.1 325 3-hydroxy-3-methylglutaryl-Coenzyme A lyase, isoform CRA_a [Rattus norvegicus]
GI:13242293 SwissProt P97519.1 325 RecName: Full=Hydroxymethylglutaryl-CoA lyase, mitochondrial; Short=HL; Short=HMG-CoA lyase; AltName: Full=3-hydroxy-3-methylglutarate-CoA lyase; Flags: Precursor [Rattus norvegicus]

Related Sequences to LMP012745 proteins

Reference Database Accession Length Protein Name
GI:13242293 EMBL CAA71148.1 325 3-hydroxy-3-methylglutaryl CoA lyase [Rattus norvegicus]
GI:13242293 GenBank AAH61797.1 325 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase [Rattus norvegicus]
GI:13242293 SwissProt P97519.1 325 RecName: Full=Hydroxymethylglutaryl-CoA lyase, mitochondrial; Short=HL; Short=HMG-CoA lyase; AltName: Full=3-hydroxy-3-methylglutarate-CoA lyase; Flags: Precursor [Rattus norvegicus]