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Overview

Uniprot IDP97519
Protein NameHydroxymethylglutaryl-CoA lyase, mitochondrial
Gene NameHmgcl
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
137 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 KGFEEAVAAG 130 AKEVSIFGAA 140 SELFTRKNVN 150 CSIEESFQRF 160 DGVMQAARAA 170 SISVRGYVSC 180 ALGCPYEGKV 190 SPAKVAEVAK 200 KLYSMGCYEI 210 SLGDTIGVGT 220 PGLMKDMLTA 230 VLHEVPVAAL 240 AVHCHDTYGQ 250 ALANTLVALQ 260 MGVSVVDSSV 270 AGLGGCPYAK 280 GASGNLATED 290 LVYMLTGLGI 300 HTGVNLQKLL 310 EAGDFICQAL 320 NRKTSSKVAQ ATCKL

Gene Ontology

Classification GO ID Description
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:0001889 liver development
Biological Process GO:0007005 mitochondrion organization
Biological Process GO:0070542 response to fatty acid
Biological Process GO:0007584 response to nutrient
Biological Process GO:0042594 response to starvation

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.