CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-006555
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Hydroxymethylglutaryl-CoA lyase, mitochondrial 
Protein Synonyms/Alias
 HL; HMG-CoA lyase; 3-hydroxy-3-methylglutarate-CoA lyase 
Gene Name
 HMGCL 
Gene Synonyms/Alias
  
Created Date
 July 27, 2013 
Organism
 Homo sapiens (Human) 
NCBI Taxa ID
 9606 
Lysine Modification
Position
Peptide
Type
References
48RDGLQNEKNIVSTPVacetylation[1, 2]
93GDHTEVLKGIQKFPGacetylation[1]
Reference
 [1] Lysine acetylation targets protein complexes and co-regulates major cellular functions.
 Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M.
 Science. 2009 Aug 14;325(5942):834-40. [PMID: 19608861]
 [2] Regulation of cellular metabolism by protein lysine acetylation.
 Zhao S, Xu W, Jiang W, Yu W, Lin Y, Zhang T, Yao J, Zhou L, Zeng Y, Li H, Li Y, Shi J, An W, Hancock SM, He F, Qin L, Chin J, Yang P, Chen X, Lei Q, Xiong Y, Guan KL.
 Science. 2010 Feb 19;327(5968):1000-4. [PMID: 20167786] 
Functional Description
 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. 
Sequence Annotation
 MOTIF 323 325 Microbody targeting signal (Potential).
 ACT_SITE 266 266 By similarity.
 METAL 42 42 Divalent metal cation.
 METAL 233 233 Divalent metal cation.
 METAL 235 235 Divalent metal cation.
 METAL 275 275 Divalent metal cation.
 BINDING 41 41 Substrate.
 MOD_RES 48 48 N6-acetyllysine.
 DISULFID 323 323 Interchain.  
Keyword
 3D-structure; Acetylation; Alternative splicing; Complete proteome; Disease mutation; Disulfide bond; Lyase; Metal-binding; Mitochondrion; Peroxisome; Reference proteome; Transit peptide. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 325 AA 
Protein Sequence
MAAMRKALPR RLVGLASLRA VSTSSMGTLP KRVKIVEVGP RDGLQNEKNI VSTPVKIKLI 60
DMLSEAGLSV IETTSFVSPK WVPQMGDHTE VLKGIQKFPG INYPVLTPNL KGFEAAVAAG 120
AKEVVIFGAA SELFTKKNIN CSIEESFQRF DAILKAAQSA NISVRGYVSC ALGCPYEGKI 180
SPAKVAEVTK KFYSMGCYEI SLGDTIGVGT PGIMKDMLSA VMQEVPLAAL AVHCHDTYGQ 240
ALANTLMALQ MGVSVVDSSV AGLGGCPYAQ GASGNLATED LVYMLEGLGI HTGVNLQKLL 300
EAGNFICQAL NRKTSSKVAQ ATCKL 325 
Gene Ontology
 GO:0005743; C:mitochondrial inner membrane; IEA:Compara.
 GO:0005759; C:mitochondrial matrix; NAS:UniProtKB.
 GO:0005777; C:peroxisome; IDA:UniProtKB.
 GO:0031406; F:carboxylic acid binding; IEA:Compara.
 GO:0000062; F:fatty-acyl-CoA binding; IEA:Compara.
 GO:0004419; F:hydroxymethylglutaryl-CoA lyase activity; IDA:UniProtKB.
 GO:0000287; F:magnesium ion binding; IDA:UniProtKB.
 GO:0030145; F:manganese ion binding; IDA:UniProtKB.
 GO:0042803; F:protein homodimerization activity; IDA:UniProtKB.
 GO:0006637; P:acyl-CoA metabolic process; IEA:Compara.
 GO:0044255; P:cellular lipid metabolic process; TAS:Reactome.
 GO:0009790; P:embryo development; IEA:Compara.
 GO:0046951; P:ketone body biosynthetic process; IDA:UniProtKB.
 GO:0006552; P:leucine catabolic process; NAS:UniProtKB.
 GO:0001889; P:liver development; IEA:Compara.
 GO:0007005; P:mitochondrion organization; IEA:Compara.
 GO:0051262; P:protein tetramerization; IDA:UniProtKB.
 GO:0070542; P:response to fatty acid; IEA:Compara.
 GO:0007584; P:response to nutrient; IEA:Compara.
 GO:0042594; P:response to starvation; IEA:Compara. 
Interpro
 IPR013785; Aldolase_TIM.
 IPR027167; HMG-CoA_lyase.
 IPR000138; HMG_CoA_lyase_AS.
 IPR000891; PYR_CT. 
Pfam
 PF00682; HMGL-like 
SMART
  
PROSITE
 PS01062; HMG_COA_LYASE
 PS50991; PYR_CT 
PRINTS