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Protein Ontology report - methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
PR:000010233 - http://purl.obolibrary.org/obo/PR_000010233
 
  Protein Forms  Complex  Annotations      
Ontology Information Show OBO stanza
  PRO ID
PR:000010233   
  PRO namemethylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial 
  Synonyms
PRO-Short-label: EXACT:MCCC1
Gene-based: RELATED:MCCA
Other: EXACT:3-methylcrotonyl-CoA carboxylase 1 | 3-methylcrotonyl-CoA carboxylase biotin-containing subunit | 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha | MCCase subunit alpha
  Definition"A protein that is a translation product of the human MCCC1 gene or a 1:1 ortholog thereof." [PRO:DNx] 
  PRO Categorygene 
  ParentPR:000000001 protein
  Terms by PRO Category
Organism-Independent Organism-Specific
         Category          Number of Terms          Category          Number of Terms
         Gene1                                                  Organism-Gene 5                                        
         Sequence1                                                  Organism-Sequence 8                                        
         Modification2                                                  Organism-Modification 4                                        
  Term Hierarchy
  Visualization
DAG: OLS:
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Protein Forms Switch to Compact View   

Category  
PRO ID
Name
Short Name
Definition
Ann. Has Annotation?

comp. In Complex?
gene


PR:000010233
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
MCCC1
"A protein that is a translation product of the human MCCC1 gene or a 1:1 ortholog thereof." [PRO:DNx]

organism-gene


PR:O04983
biotin carboxylase, chloroplastic (Arabidopsis thaliana)
At-MCCC1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of Arabidopsis thaliana." [OMA:O04983, PRO:DNx]

organism-sequence


PR:O04983-1
biotin carboxylase, chloroplastic isoform 1 (Arabidopsis thaliana)
At-MCCC1/iso:1
"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]

organism-gene


PR:Q54KE6
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (Dictyostelium discoideum)
Dicty-MCCC1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of Dictyostelium discoideum." [OMA:Q54KE6, PRO:DNx]

organism-gene


PR:Q5I0C3
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (rat)
rMCCC1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of rat." [OMA:Q5I0C3, PRO:DNx]

organism-gene


PR:Q96RQ3
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human)
hMCCC1
"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]

organism-sequence


PR:000026110
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant (human)
hMCCC1/var+
"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]

organism-sequence


PR:000026111
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant MCCA D532H (human)
hMCCC1/var:MCCA D532H
"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]
Yes
organism-sequence


PR:000026112
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant MCCA L437P (human)
hMCCC1/var:MCCA L437P
"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]
Yes
organism-sequence


PR:000026113
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant MCCA R385S (human)
hMCCC1/var:MCCA R385S
"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]
Yes
organism-sequence


PR:000026114
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant MCCA A289V (human)
hMCCC1/var:MCCA A289V
"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]
Yes
organism-sequence


PR:Q96RQ3-1
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (human)
hMCCC1/iso:1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that is encoded in the genome of human." [PMID:11401427, PRO:CNA]

organism-modification


PR:000048349
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed form (human)
hMCCC1/TransPep-
"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]

organism-modification


PR:000048350
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed biotinylated 1 (human)
hMCCC1/TransPep-/Btn:1
"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]

organism-modification


PR:000068018
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial phosphorylated 1 (human)
hMCCC1/Phos:1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) that has been phosphorylated on the residue at the position equivalent to Tyr-270 of the amino acid sequence represented by UniProtKB:Q96RQ3. UniProtKB:Q96RQ3, Tyr-270, MOD:00048." [IEDB_epitope:2145918, PRO:DNx]

organism-modification


PR:000068242
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial cysteinylated 1 (human)
hMCCC1/Cyst:1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (human) that has been cysteinylated on the residue at the position equivalent to Cys-595 of the amino acid sequence represented by UniProtKB:Q96RQ3. UniProtKB:Q96RQ3, Cys-595, MOD:00765." [IEDB_epitope:1582297, PRO:DNx]

organism-gene


PR:Q99MR8
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (mouse)
mMCCC1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that is encoded in the genome of mouse." [OMA:Q99MR8, PRO:CJB]

Yes
organism-sequence


PR:Q99MR8-1
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (mouse)
mMCCC1/iso:1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that is encoded in the genome of mouse." [PMID:11181649, PRO:CNA]

sequence


PR:000026106
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1
MCCC1/iso:1
"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]

organism-sequence


PR:Q96RQ3-1
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (human)
hMCCC1/iso:1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that is encoded in the genome of human." [PMID:11401427, PRO:CNA]

organism-sequence


PR:Q99MR8-1
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (mouse)
mMCCC1/iso:1
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that is encoded in the genome of mouse." [PMID:11181649, PRO:CNA]

modification


PR:000026695
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1, transit peptide removed form
MCCC1/iso:1/TransPep-
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that has had the transit peptide removed." [PRO:DNx]

modification


PR:000026694
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed form
MCCC1/TransPep-
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial that has had the transit peptide removed." [PRO:DNx]

modification


PR:000026695
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1, transit peptide removed form
MCCC1/iso:1/TransPep-
"A methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 that has had the transit peptide removed." [PRO:DNx]

organism-modification


PR:000048349
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed form (human)
hMCCC1/TransPep-
"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]

organism-modification


PR:000048350
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed biotinylated 1 (human)
hMCCC1/TransPep-/Btn:1
"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]

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MCCC1 forms found in complexes   

MCCC1 Component
 Complexes
PR:Q99MR8 mMCCC1 PR:000025760 methylcrotonoyl-CoA carboxylase complex, mitochondrial (mouse)
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Functional Annotation (PRO-centric view) Switch to GO Centric View   
PRO Term GO Annotation Evidence
PR:000026111 hMCCC1/var:MCCA D532H associated_with_disease_progressionDO:0050710 3-methylcrotonyl-CoA carboxylase deficiency PMID:11181649
located_inSO:0001630 splice_region_variant PMID:11181649
PR:000026112 hMCCC1/var:MCCA L437P
UniProtKB:Q96RQ3, Leu-437, CHEBI:50342		 associated_with_disease_progressionDO:0050710 3-methylcrotonyl-CoA carboxylase deficiency PMID:11181649
located_inSO:0001559 polypeptide_loss_of_function_variant PMID:11181649
PR:000026113 hMCCC1/var:MCCA R385S
UniProtKB:Q96RQ3, Arg-385, CHEBI:29999		 associated_with_disease_progressionDO:0050710 3-methylcrotonyl-CoA carboxylase deficiency PMID:11181649
located_inSO:0001559 polypeptide_loss_of_function_variant PMID:11181649
PR:000026114 hMCCC1/var:MCCA A289V
UniProtKB:Q96RQ3, Ala-289, CHEBI:30015		 associated_with_disease_progressionDO:0050710 3-methylcrotonyl-CoA carboxylase deficiency PMID:11181649
located_inSO:0001559 polypeptide_loss_of_function_variant PMID:11181649