Search Results

Overview

Uniprot IDP35914
Protein NameHydroxymethylglutaryl-CoA lyase, mitochondrial
Gene NameHMGCL
OrganismHomo sapiens

Kla Sites from experimental identification

Position Flanking peptide
137 ASELFTKKNINCSIE
179 LGCPYEGKISPAKVA
184 EGKISPAKVAEVTKK
317 LNRKTSSKVAQATCK
34 GTLPKRVKIVEVGPR
48 RDGLQNEKNIVSTPV
93 GDHTEVLKGIQKFPG

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 MAAMRKALPR 20 RLVGLASLRA 30 VSTSSMGTLP 40 KRVKIVEVGP 50 RDGLQNEKNI 60 VSTPVKIKLI 70 DMLSEAGLSV 80 IETTSFVSPK 90 WVPQMGDHTE 100 VLKGIQKFPG 110 INYPVLTPNL 120 KGFEAAVAAG 130 AKEVVIFGAA 140 SELFTKKNIN 150 CSIEESFQRF 160 DAILKAAQSA 170 NISVRGYVSC 180 ALGCPYEGKI 190 SPAKVAEVTK 200 KFYSMGCYEI 210 SLGDTIGVGT 220 PGIMKDMLSA 230 VMQEVPLAAL 240 AVHCHDTYGQ 250 ALANTLMALQ 260 MGVSVVDSSV 270 AGLGGCPYAQ 280 GASGNLATED 290 LVYMLEGLGI 300 HTGVNLQKLL 310 EAGNFICQAL 320 NRKTSSKVAQ ATCKL

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005829 cytosol
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0005739 mitochondrion
Cellular Component GO:0005782 peroxisomal matrix
Cellular Component GO:0005777 peroxisome
Cellular Component GO:0032991 protein-containing complex
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:0046951 ketone body biosynthetic process
Biological Process GO:0006552 L-leucine catabolic process
Biological Process GO:0006629 lipid metabolic process

Reference

[1] Yang Z, Yan C, Ma J, Peng P, Ren X et al.. Lactylome analysis suggests lactylation-dependent mechanisms of metabolic adaptation in hepatocellular carcinoma.. Nat Metab 5(1):61-79. 2023 Jan. PMID: 36593272.

[2] Hong H, Chen X, Wang H, Gu X, Yuan Y et al.. Global profiling of protein lysine lactylation and potential target modified protein analysis in hepatocellular carcinoma.. Proteomics 23(9):e2200432. 2023 May. PMID: 36625413.

[3] Shi CM, Wang QC, Li XL, Yang YH, Tang XY et al.. Global Profiling of Protein Lactylation in Human Hippocampi.. Proteomics Clin Appl 19(2):e202400061. 2025 Mar. PMID: 39610256.