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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 9                                        
  Term Hierarchy
  Visualization
DAG: OLS:
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Protein Forms  Switch to Extended View   

Category  
PRO Form Name
Short Name
Ann. Has Annotation?

Comp. In Complex?
gene


PR:000010233 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
MCCC1

organism-gene


PR:O04983 biotin carboxylase, chloroplastic (Arabidopsis thaliana)
At-MCCC1

organism-sequence


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

organism-gene


PR:Q54KE6 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (Dictyostelium discoideum)
Dicty-MCCC1

organism-gene


PR:Q5I0C3 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (rat)
rMCCC1

organism-gene


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

organism-sequence


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

organism-sequence


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


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


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


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


PR:Q96RQ3-1 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (human)
hMCCC1/iso:1

organism-modification


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

organism-modification


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

organism-modification


PR:000085804 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant D532H, transit peptide removed biotinylated 1 (human)
hMCCC1/var:D532H/TransPep-/Btn:1

organism-modification


PR:000085840 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant M325R, transit peptide removed biotinylated 1 (human)
hMCCC1/var:M325R/TransPep-/Btn:1

organism-modification


PR:000085950 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant L437P, transit peptide removed biotinylated 1 (human)
hMCCC1/var:L437P/TransPep-/Btn:1

organism-modification


PR:000085954 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant I460M, transit peptide removed biotinylated 1 (human)
hMCCC1/var:I460M/TransPep-/Btn:1

organism-modification


PR:000085987 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant R385S, transit peptide removed biotinylated 1 (human)
hMCCC1/var:R385S/TransPep-/Btn:1

organism-modification


PR:000068018 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial phosphorylated 1 (human)
hMCCC1/Phos:1

organism-modification


PR:000068242 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial cysteinylated 1 (human)
hMCCC1/Cyst:1

organism-gene


PR:Q99MR8 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (mouse)
mMCCC1

Yes
organism-sequence


PR:Q99MR8-1 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (mouse)
mMCCC1/iso:1

sequence


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

organism-sequence


PR:Q96RQ3-1 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (human)
hMCCC1/iso:1

organism-sequence


PR:Q99MR8-1 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 (mouse)
mMCCC1/iso:1

modification


PR:000026695 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1, transit peptide removed form
MCCC1/iso:1/TransPep-

modification


PR:000026694 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, transit peptide removed form
MCCC1/TransPep-

modification


PR:000026695 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1, transit peptide removed form
MCCC1/iso:1/TransPep-

organism-modification


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

organism-modification


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

organism-modification


PR:000085804 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant D532H, transit peptide removed biotinylated 1 (human)
hMCCC1/var:D532H/TransPep-/Btn:1

organism-modification


PR:000085840 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant M325R, transit peptide removed biotinylated 1 (human)
hMCCC1/var:M325R/TransPep-/Btn:1

organism-modification


PR:000085950 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant L437P, transit peptide removed biotinylated 1 (human)
hMCCC1/var:L437P/TransPep-/Btn:1

organism-modification


PR:000085954 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant I460M, transit peptide removed biotinylated 1 (human)
hMCCC1/var:I460M/TransPep-/Btn:1

organism-modification


PR:000085987 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial sequence variant R385S, transit peptide removed biotinylated 1 (human)
hMCCC1/var:R385S/TransPep-/Btn:1

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MCCC1 forms found in complexes   

MCCC1 Component
 Complexes
PR:Q99MR8 mMCCC1 PR:000025760 methylcrotonoyl-CoA carboxylase complex (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