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Overview

Uniprot IDQ5I0K3
Protein NameCitramalyl-CoA lyase, mitochondrial
Gene NameClybl
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
80 EDGVAENKKNEARLR

Function

Mitochondrial enzyme required to detoxify vitamin B12-poisoning metabolites. Acts as a citramalyl-CoA lyase by converting citramalyl-CoA into acetyl-CoA and pyruvate in the C5-dicarboxylate catabolism pathway, a pathway required to detoxify itaconate, a vitamin B12-poisoning metabolite. Also acts as a malyl-CoA thioesterase to detoxify malyl-CoA, a side product of citric acid cycle enzymes, which is toxic for the vitamin B12-dependent enzyme MMUT. Also acts as a malate synthase in vitro, converting glyoxylate and acetyl-CoA to malate. Also acts as a beta-methylmalate synthase in vitro, by mediating conversion of glyoxylate and propionyl-CoA to beta-methylmalate. Also has very weak citramalate synthase activity in vitro

Protein Sequence

10 MALCVLQNAV 20 RGAAALPRLK 30 ASLVASVCRP 40 GYSSLSNHKY 50 VPRRAVLYVP 60 GNDEKKIRKI 70 PSLKVDCAVL 80 DCEDGVAENK 90 KNEARLRIAK 100 TLEDFDLGTT 110 EKCVRINSVS 120 SGLAEADLET 130 FLQARVLPSS 140 LMLPKVEGPE 150 EIQWFSDKFS 160 LHLKGRKLEQ 170 PMNLIPFVET 180 AMGLLNFKAV 190 CEETLKIGPQ 200 VGLFLDAVVF 210 GGEDFRASIG 220 ATSNKDTQDI 230 LYARQKIVVT 240 AKAFGLQAID 250 LVYIDFRDED 260 GLLRQSREAA 270 AMGFTGKQVI 280 HPNQIAVVQE 290 QFTPTPEKIR 300 WAEELIAAFK 310 EHQQLGKGAF 320 TFQGSMIDMP 330 LLKQAQNIVT LATSIKEK

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0047777 (S)-citramalyl-CoA lyase activity
Molecular Function GO:0016289 acyl-CoA hydrolase activity
Molecular Function GO:0000287 magnesium ion binding
Molecular Function GO:0004474 malate synthase activity
Biological Process GO:0106121 positive regulation of cobalamin metabolic process
Biological Process GO:0070207 protein homotrimerization
Biological Process GO:0106064 regulation of cobalamin metabolic process
Biological Process GO:0110052 toxic metabolite repair

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.