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Overview

Uniprot IDA0A8L2Q6E3
Protein NameHydroxymethylglutaryl-CoA lyase, mitochondrial
Gene NameHmgcl
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
136 ASELFTRKNVNCSIE

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 MATVRKAFPQ 20 RLVGLASLRA 30 ASTSSMGTLP 40 KRVKIVEVGP 50 RDGLQNEKSI 60 VPTPVKIKLI 70 DMLSEAGLPV 80 IEATSFVSPK 90 WVPQMADHSD 100 VLKGIQKFPG 110 INYPVLTPNM 120 KGFEEAVRGA 130 KEVSIFGAAS 140 ELFTRKNVNC 150 SIEESFQRFD 160 GVMQAARAAS 170 ISVRGYVSCA 180 LGCPYEGKVS 190 PAKVAEVAKK 200 LYSMGCYEIS 210 LGDTIGVGTP 220 GLMKDMLTAV 230 LHEVPVAALA 240 VHCHDTYGQA 250 LANTLVALQM 260 GVSVVDSSVA 270 GLGGCPYAKG 280 ASGNLATEDL 290 VYMLTGLGIH 300 TGVNLQKLLE 310 AGDFICQALN 320 RKTSSKVAQA TCKL

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0005777 peroxisome
Molecular Function GO:0004419 hydroxymethylglutaryl-CoA lyase activity
Molecular Function GO:0046872 metal ion binding
Biological Process GO:0006629 lipid metabolic process

Reference

[1] Chen Y, Sun W, Sun Z, Zhao H, Wu T et al.. Effect of electroacupuncture on hippocampal protein lactylation in a rat model of vascular dementia.. Front Neurol 16:1629474. 2025. PMID: 40963935.