Search Results

Overview

Uniprot ID	P38060
Protein Name	Hydroxymethylglutaryl-CoA lyase, mitochondrial
Gene Name	Hmgcl
Organism	Mus musculus

Kla Sites from experimental identification

Position	Flanking peptide
137	VSELFTRKNANCSIE
48	RDGLQNEKSIVPTPV

Function

Mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. 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

Protein Sequence

10 MASVRKAFPR 20 RLVGLTSLRA 30 VSTSSMGTLP 40 KQVKIVEVGP 50 RDGLQNEKSI 60 VPTPVKIRLI 70 DMLSEAGLPV 80 IEATSFVSPK 90 WVPQMADHSD 100 VLKGIQKFPG 110 INYPVLTPNM 120 KGFEEAVAAG 130 AKEVSVFGAV 140 SELFTRKNAN 150 CSIEESFQRF 160 AGVMQAAQAA 170 SISVRGYVSC 180 ALGCPYEGKV 190 SPAKVAEVAK 200 KLYSMGCYEI 210 SLGDTIGVGT 220 PGLMKDMLTA 230 VMHEVPVTAL 240 AVHCHDTYGQ 250 ALANTLVALQ 260 MGVSVVDSSV 270 AGLGGCPYAK 280 GASGNLATED 290 LVYMLNGLGI 300 HTGVNLQKLL 310 EAGDFICQAL 320 NRKTSSKVAQ ATCKL

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0005743	mitochondrial inner membrane
Cellular Component	GO:0005759	mitochondrial matrix
Cellular Component	GO:0005739	mitochondrion
Cellular Component	GO:0005777	peroxisome
Cellular Component	GO:0032991	protein-containing complex
Molecular Function	GO:0031406	carboxylic acid binding
Molecular Function	GO:0000062	fatty-acyl-CoA binding
Molecular Function	GO:0004419	hydroxymethylglutaryl-CoA lyase activity
Molecular Function	GO:0000287	magnesium ion binding
Molecular Function	GO:0030145	manganese ion binding
Molecular Function	GO:0046872	metal ion binding
Molecular Function	GO:0005198	structural molecule activity
Biological Process	GO:0006637	acyl-CoA metabolic process
Biological Process	GO:0046951	ketone body biosynthetic process
Biological Process	GO:0006552	L-leucine catabolic process
Biological Process	GO:0006629	lipid metabolic process
Biological Process	GO:0007005	mitochondrion organization

Reference

[1] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[2] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.