[Term] id: PR:Q54KE6 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (Dictyostelium discoideum) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of Dictyostelium discoideum." [OMA:Q54KE6, PANTHER:PTHR18866:SF17, PRO:DNx] comment: Category=organism-gene. synonym: "mccA" RELATED Gene-based [UniProtKB:Q54KE6] synonym: "mccc1" RELATED Gene-based [UniProtKB:Q54KE6] synonym: "Dicty-MCCC1" EXACT PRO-short-label [PRO:DNx] synonym: "DDB_G0287377" RELATED Gene-based [UniProtKB:Q54KE6] synonym: "MCCase subunit alpha (Dictyostelium discoideum)" EXACT [UniProtKB:Q54KE6] synonym: "3-methylcrotonyl-CoA carboxylase 1 (Dictyostelium discoideum)" EXACT [UniProtKB:Q54KE6] synonym: "3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha (Dictyostelium discoideum)" EXACT [UniProtKB:Q54KE6] synonym: "3-methylcrotonyl-CoA carboxylase biotin-containing subunit (Dictyostelium discoideum)" EXACT [UniProtKB:Q54KE6] xref: UniProtKB:Q54KE6 intersection_of: only_in_taxon NCBITaxon:44689 ! Dictyostelium discoideum intersection_of: PR:000010233 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial relationship: has_gene_template dictyBase:DDB_G0287377 ! mccA (Dictyostelium discoideum) [Term] id: PR:O04983-1 name: biotin carboxylase, chloroplastic isoform 1 (Arabidopsis thaliana) def: "A biotin carboxylase, chloroplastic (Arabidopsis thaliana) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:O04983-1." [PRO:DNx, UniProtKB:O04983] comment: Category=organism-sequence. synonym: "At-MCCC1/iso:1" EXACT PRO-short-label [PRO:DNx] xref: UniProtKB:O04983-1 is_a: PR:O04983 ! biotin carboxylase, chloroplastic (Arabidopsis thaliana) relationship: only_in_taxon NCBITaxon:3702 ! Arabidopsis thaliana [Term] id: PR:Q96RQ3-1 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that is encoded in the genome of human." [PMID:11401427, PRO:CNA] comment: Category=organism-sequence. synonym: "hMCCC1/iso:1" EXACT PRO-short-label [PRO:DNx] xref: UniProtKB:Q96RQ3-1 intersection_of: only_in_taxon NCBITaxon:9606 ! Homo sapiens intersection_of: PR:000026106 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 [Term] id: PR:000010233 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial def: "A protein that is a translation product of the human MCCC1 gene or a 1:1 ortholog thereof." [PRO:DNx] comment: Category=gene. synonym: "MCCA" RELATED Gene-based [] synonym: "MCCC1" EXACT PRO-short-label [PRO:DNx] synonym: "MCCase subunit alpha" EXACT [] synonym: "3-methylcrotonyl-CoA carboxylase 1" EXACT [] synonym: "3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha" EXACT [] synonym: "3-methylcrotonyl-CoA carboxylase biotin-containing subunit" EXACT [] is_a: PR:000000001 ! protein [Term] id: PR:000026114 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant MCCA A289V (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) that has a Val residue at the position equivalent to Ala-289 of the amino acid sequence represented by UniProtKB:Q96RQ3. UniProtKB:Q96RQ3, Ala-289, CHEBI:30015." [PMID:11181649, PRO:CNA, PRO:DAN] comment: Category=organism-sequence. synonym: "hMCCC1/var:MCCA A289V" EXACT PRO-short-label [PRO:DNx] synonym: "UniProtKB:Q96RQ3, Ala-289, CHEBI:30015" EXACT PRO-proteoform-std [PRO:DAN] xref: UniProtKB_VAR:VAR_012785 is_a: PR:000026110 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:Q96RQ3 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of human. The human MCCC1 gene contains 19 exons, and maps to the chromosomal location 3q25-27." [PMID:11170888, PRO:CNA] comment: Category=organism-gene. synonym: "MCCA" RELATED Gene-based [UniProtKB:Q96RQ3] synonym: "MCCC1" RELATED Gene-based [UniProtKB:Q96RQ3] synonym: "hMCCC1" EXACT PRO-short-label [PRO:DNx] synonym: "MCCase subunit alpha (human)" EXACT [UniProtKB:Q96RQ3] synonym: "3-methylcrotonyl-CoA carboxylase 1 (human)" EXACT [UniProtKB:Q96RQ3] synonym: "3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha (human)" EXACT [UniProtKB:Q96RQ3] synonym: "3-methylcrotonyl-CoA carboxylase biotin-containing subunit (human)" EXACT [UniProtKB:Q96RQ3] xref: UniProtKB:Q96RQ3 intersection_of: only_in_taxon NCBITaxon:9606 ! Homo sapiens intersection_of: PR:000010233 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial relationship: has_gene_template HGNC:6936 ! MCCC1 (human) [Term] id: PR:000026112 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant MCCA L437P (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) that has a Pro residue at the position equivalent to Leu-437 of the amino acid sequence represented by UniProtKB:Q96RQ3. UniProtKB:Q96RQ3, Leu-437, CHEBI:50342." [PMID:11181649, PRO:CNA, PRO:DAN] comment: Category=organism-sequence. synonym: "hMCCC1/var:MCCA L437P" EXACT PRO-short-label [PRO:DNx] synonym: "UniProtKB:Q96RQ3, Leu-437, CHEBI:50342" EXACT PRO-proteoform-std [PRO:DAN] xref: UniProtKB_VAR:VAR_012788 is_a: PR:000026110 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:000026113 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant MCCA R385S (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) that has a Ser residue at the position equivalent to Arg-385 of the amino acid sequence represented by UniProtKB:Q96RQ3. UniProtKB:Q96RQ3, Arg-385, CHEBI:29999." [PMID:11181649, PRO:CNA, PRO:DAN] comment: Category=organism-sequence. synonym: "hMCCC1/var:MCCA R385S" EXACT PRO-short-label [PRO:DNx] synonym: "UniProtKB:Q96RQ3, Arg-385, CHEBI:29999" EXACT PRO-proteoform-std [PRO:DAN] xref: UniProtKB_VAR:VAR_012787 is_a: PR:000026110 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:000026110 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) that has at least one amino acid residue that differs from the corresponding residue found in another methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) when such a difference arises as the result of genetic differences, i.e., different alleles of methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human). Operationally, this means the sequence representation of the methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) has at least one amino acid residue that differs from the equivalent residue found in the sequence represented by UniProtKB:Q96RQ3-1." [PRO:CNA] comment: Category=organism-sequence. synonym: "hMCCC1/var+" EXACT PRO-short-label [PRO:DNx] is_a: PR:Q96RQ3 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:000026111 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant MCCA D532H (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) that lacks the region encoded by exon 13 and shifts the reading frame due to the missense mutation D532H altering the splice site at the position equivalent to Asp-532 of the amino acid sequence represented by UniProtKB:Q96RQ3." [PMID:11181649, PRO:CNA, PRO:DAN] comment: Category=organism-sequence. Note: The mutation indicated also results in its removal after splicing. Thus, despite the designation used in the publication (D532H), there is no position 532 in the final protein. synonym: "hMCCC1/var:MCCA D532H" EXACT PRO-short-label [PRO:DNx] is_a: PR:000026110 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human) relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:000048350 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed biotinylated 1 (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) that has had the transit peptide removed and has been post-translationally modified to include biotinylated lysine at position 681 of the amino acid sequence represented by UniProtKB:Q96RQ3. UniProtKB:Q96RQ3, 42-725, Lys-681, MOD:00126." [PRO:DNx, Reactome:R-HSA-3323154, Reactome:R-HSA-70748] comment: Category=organism-modification. synonym: "hMCCC1/TransPep-/Btn:1" EXACT PRO-short-label [PRO:DNx] synonym: "PRO_0000002833, Lys-681, MOD:00126" EXACT PRO-proteoform-ftid [PRO:DNx] synonym: "UniProtKB:Q96RQ3, 42-725, Lys-681, MOD:00126" EXACT PRO-proteoform-std [PRO:DNx] xref: Reactome:R-HSA-70748 "PR:000048350 located_in GO:0005759" xref: Reactome:R-HSA-3323154 "PR:000048350 located_in GO:0005829" is_a: PR:Q96RQ3 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) relationship: has_part MOD:00126 ! N6-biotinyl-L-lysine relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens relationship: lacks_part SO:0000725 ! transit_peptide [Term] id: PR:Q99MR8 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (mouse) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of mouse." [OMA:Q99MR8, PRO:CJB] comment: Category=organism-gene. synonym: "Mcca" RELATED Gene-based [UniProtKB:Q99MR8] synonym: "Mccc1" RELATED Gene-based [UniProtKB:Q99MR8] synonym: "mMCCC1" EXACT PRO-short-label [PRO:DNx] synonym: "MCCase subunit alpha (mouse)" EXACT [UniProtKB:Q99MR8] synonym: "3-methylcrotonyl-CoA carboxylase 1 (mouse)" EXACT [UniProtKB:Q99MR8] synonym: "3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha (mouse)" EXACT [UniProtKB:Q99MR8] synonym: "3-methylcrotonyl-CoA carboxylase biotin-containing subunit (mouse)" EXACT [UniProtKB:Q99MR8] xref: UniProtKB:Q99MR8 intersection_of: only_in_taxon NCBITaxon:10090 ! Mus musculus intersection_of: PR:000010233 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial relationship: has_gene_template MGI:1919289 ! Mccc1 (mouse) [Term] id: PR:Q5I0C3 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (rat) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of rat." [OMA:Q5I0C3, PANTHER:PTHR18866:SF17, PRO:DNx] comment: Category=organism-gene. synonym: "Mcca" RELATED Gene-based [UniProtKB:Q5I0C3] synonym: "Mccc1" RELATED Gene-based [UniProtKB:Q5I0C3] synonym: "rMCCC1" EXACT PRO-short-label [PRO:DNx] synonym: "MCCase subunit alpha (rat)" EXACT [UniProtKB:Q5I0C3] synonym: "3-methylcrotonyl-CoA carboxylase 1 (rat)" EXACT [UniProtKB:Q5I0C3] synonym: "3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha (rat)" EXACT [UniProtKB:Q5I0C3] synonym: "3-methylcrotonyl-CoA carboxylase biotin-containing subunit (rat)" EXACT [UniProtKB:Q5I0C3] xref: UniProtKB:Q5I0C3 intersection_of: only_in_taxon NCBITaxon:10116 ! Rattus norvegicus intersection_of: PR:000010233 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial relationship: has_gene_template RGD:1310615 ! Mccc1 (rat) [Term] id: PR:Q99MR8-1 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (mouse) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that is encoded in the genome of mouse." [PMID:11181649, PRO:CNA] comment: Category=organism-sequence. synonym: "mMCCC1/iso:1" EXACT PRO-short-label [PRO:DNx] xref: UniProtKB:Q99MR8-1 intersection_of: only_in_taxon NCBITaxon:10090 ! Mus musculus intersection_of: PR:000026106 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 [Term] id: PR:000048349 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed form (human) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) that has had the transit peptide removed. UniProtKB:Q96RQ3, 42-725." [PRO:DNx, Reactome:R-HSA-3323164] comment: Category=organism-modification. synonym: "PRO_0000002833" EXACT PRO-proteoform-ftid [PRO:DNx] synonym: "hMCCC1/TransPep-" EXACT PRO-short-label [PRO:DNx] synonym: "UniProtKB:Q96RQ3, 42-725" EXACT PRO-proteoform-std [PRO:DNx] xref: Reactome:R-HSA-3323164 "PR:000048349 located_in GO:0005829" intersection_of: PR:Q96RQ3 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) intersection_of: lacks_part SO:0000725 ! transit_peptide relationship: only_in_taxon NCBITaxon:9606 ! Homo sapiens [Term] id: PR:000026106 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:Q96RQ3-1 or a 1:1 ortholog thereof." [PMID:11401427, PRO:CNA] comment: Category=sequence. synonym: "MCCC1/iso:1" EXACT PRO-short-label [PRO:DNx] is_a: PR:000010233 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [Term] id: PR:000026695 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1, transit peptide removed form def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that has had the transit peptide removed." [PRO:DNx] comment: Category=modification. synonym: "MCCC1/iso:1/TransPep-" EXACT PRO-short-label [PRO:DNx] intersection_of: PR:000026106 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 intersection_of: lacks_part SO:0000725 ! transit_peptide [Term] id: PR:000026694 name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed form def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that has had the transit peptide removed." [PRO:DNx] comment: Category=modification. synonym: "MCCC1/TransPep-" EXACT PRO-short-label [PRO:DNx] intersection_of: PR:000010233 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial intersection_of: lacks_part SO:0000725 ! transit_peptide [Term] id: PR:O04983 name: biotin carboxylase, chloroplastic (Arabidopsis thaliana) def: "A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of Arabidopsis thaliana." [OMA:O04983, PRO:DNx] comment: Category=organism-gene. synonym: "CAC2" RELATED Gene-based [UniProtKB:O04983] synonym: "At-MCCC1" EXACT PRO-short-label [PRO:DNx] synonym: "T26D22.8" RELATED Gene-based [UniProtKB:O04983] synonym: "At5g35360" RELATED Gene-based [UniProtKB:O04983] synonym: "acetyl-coenzyme A carboxylase biotin carboxylase subunit A (Arabidopsis thaliana)" EXACT [UniProtKB:O04983] xref: UniProtKB:O04983 intersection_of: only_in_taxon NCBITaxon:3702 ! Arabidopsis thaliana intersection_of: PR:000010233 ! methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial relationship: has_gene_template Araport:AT5G35360 ! CAC2 (Arabidopsis thaliana)