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Overview

Uniprot IDA0A8I6B1P6
Protein NameCitramalyl-CoA lyase, mitochondrial
Gene NameClybl
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
81 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 NLVCVVSSST 20 MWAICHFPCR 30 KASLVASVCR 40 PGYSSLSNHK 50 YVPRRAVLYV 60 PGNDEKKIRK 70 IPSLKVDCAV 80 LDCEDGVAEN 90 KKNEARLRIA 100 KTLEDFDLGT 110 TEKCVRINSV 120 SSGLAEADLE 130 TFLQARVLPS 140 SLMLPKVEGP 150 EEIQWFSDKF 160 SLHLKGRKLE 170 QPMNLIPFVE 180 TAMGLLNFKA 190 VCEETLKIGP 200 QVGLFLDAVV 210 FGGEDFRASI 220 GATSNKDTQD 230 ILYARQKIVV 240 TAKAFGLQAI 250 DLVYIDFRDE 260 DGLLRQSREA 270 AAMGFTGKQV 280 IHPNQIAVVQ 290 EQFTPTPEKI 300 RWAEELIAAF 310 KEHQQLGKGA 320 FTFQGSMIDM 330 PLLKQAQNIV TLATSIKEK

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: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.