[Term]
id: PR:000085840
name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant M325R, transit peptide removed biotinylated 1 (human)
def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) that has an Arg residue at the position equivalent to Met-325 of the amino acid sequence represented by UniProtKB:Q96RQ3, that has had the transit peptide removed, and that has been biotinylated on the residue at the position equivalent to Lys-681. UniProtKB:Q96RQ3, 42-725, Met-325, CHEBI:29952|Lys-681, MOD:00126." [PRO:DNx, Reactome:R-HSA-9908669]
comment: Category=organism-modification.
synonym: "hMCCC1/var:M325R/TransPep-/Btn:1" EXACT PRO-short-label [PRO:DNx]
synonym: "M325R Btn-MCCC1" RELATED Reactome-displayName [PRO:DNx, Reactome:R-HSA-9908669]
synonym: "UniProtKB:Q96RQ3, 42-725, Met-325, CHEBI:29952|Lys-681, MOD:00126" EXACT PRO-proteoform-std [PRO:DNx]
xref: Reactome:R-HSA-9908669 "PR:000085840 located_in GO:0005759"
is_a: PR:Q96RQ3 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human)
relationship: has_part MOD:00126 ! N6-biotinyl-L-lysine
relationship: lacks_part SO:0000725 ! transit_peptide
relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens