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HUMAN:MCCA

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Species (Taxon ID) Homo sapiens (Human). (9606)
Gene Name(s) MCCC1 (synonyms: MCCA)
Protein Name(s) Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

MCCase subunit alpha 3-methylcrotonyl-CoA carboxylase 1 3-methylcrotonyl-CoA carboxylase biotin-containing subunit 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha

External Links
UniProt Q96RQ3
EMBL AF310972
AB029826
AF297332
AF310339
AK023051
AB209737
BC004214
BC004187
CCDS CCDS3241.1
RefSeq NP_001280202.1
NP_064551.3
UniGene Hs.47649
PDB 2EJM
PDBsum 2EJM
ProteinModelPortal Q96RQ3
SMR Q96RQ3
BioGrid 121249
IntAct Q96RQ3
STRING 9606.ENSP00000265594
DrugBank DB00121
PhosphoSite Q96RQ3
DMDM 108861983
MaxQB Q96RQ3
PaxDb Q96RQ3
PRIDE Q96RQ3
Ensembl ENST00000265594
GeneID 56922
KEGG hsa:56922
UCSC uc003fle.3
CTD 56922
GeneCards GC03M182733
HGNC HGNC:6936
HPA HPA008310
MIM 210200
609010
neXtProt NX_Q96RQ3
Orphanet 6
PharmGKB PA30680
eggNOG COG4770
GeneTree ENSGT00550000074675
HOGENOM HOG000008989
HOVERGEN HBG000555
InParanoid Q96RQ3
KO K01968
OMA VDNPRHV
OrthoDB EOG7RZ5PH
PhylomeDB Q96RQ3
TreeFam TF105650
BioCyc MetaCyc:ENSG00000078070-MONOMER
Reactome REACT_11153
REACT_169312
REACT_197
UniPathway UPA00363
ChiTaRS MCCC1
EvolutionaryTrace Q96RQ3
GenomeRNAi 56922
NextBio 62428
PRO PR:Q96RQ3
Proteomes UP000005640
Bgee Q96RQ3
CleanEx HS_MCCC1
ExpressionAtlas Q96RQ3
Genevestigator Q96RQ3
GO GO:0005829
GO:0005743
GO:0005759
GO:0005739
GO:0005524
GO:0009374
GO:0004075
GO:0046872
GO:0004485
GO:0006768
GO:0009083
GO:0034641
GO:0006552
GO:0044281
GO:0006766
GO:0006767
Gene3D 3.30.1490.20
3.30.470.20
3.40.50.20
InterPro IPR011761
IPR013815
IPR013816
IPR001882
IPR011764
IPR005482
IPR000089
IPR005481
IPR005479
IPR016185
IPR011054
IPR011053
Pfam PF02785
PF00364
PF00289
PF02786
SMART SM00878
SUPFAM SSF51230
SSF51246
SSF52440
PROSITE PS50975
PS50979
PS00188
PS50968
PS00867

Annotations

Qualifier GO ID GO term name Reference ECO ID ECO term name with/from Aspect Extension Notes Status

Contributes to

GO:0004485

methylcrotonoyl-CoA carboxylase activity

PMID:17360195[1]

ECO:0000314

F

Figure 3

complete
CACAO 5310

contributes_to

GO:0004485

methylcrotonoyl-CoA carboxylase activity

PMID:11170888[2]

ECO:0000303

author statement without traceable support used in manual assertion

F

part_of:(GO:0006552)

Seeded From UniProt

complete

part_of

GO:0002169

3-methylcrotonyl-CoA carboxylase complex, mitochondrial

PMID:11170888[2]

ECO:0000303

author statement without traceable support used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:1905202

methylcrotonoyl-CoA carboxylase complex

PMID:17360195[1]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

involved_in

GO:0051291

protein heterooligomerization

PMID:11170888[2]

ECO:0000303

author statement without traceable support used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0006552

leucine catabolic process

PMID:11170888[2]

ECO:0000250

sequence similarity evidence used in manual assertion

UniProtKB:Q6T5L7

P

Seeded From UniProt

complete

part_of

GO:0002169

3-methylcrotonyl-CoA carboxylase complex, mitochondrial

PMID:11181649[3]

ECO:0000303

author statement without traceable support used in manual assertion

C

Seeded From UniProt

complete

contributes_to

GO:0004485

methylcrotonoyl-CoA carboxylase activity

PMID:11181649[3]

ECO:0000303

author statement without traceable support used in manual assertion

F

part_of:(GO:0006552)

Seeded From UniProt

complete

enables

GO:0004075

biotin carboxylase activity

PMID:22869039[4]

ECO:0000303

author statement without traceable support used in manual assertion

F

Seeded From UniProt

complete

involved_in

GO:0006768

biotin metabolic process

PMID:22869039[4]

ECO:0000303

author statement without traceable support used in manual assertion

P

Seeded From UniProt

complete

contributes_to

GO:0004485

methylcrotonoyl-CoA carboxylase activity

PMID:11401427[5]

ECO:0000303

author statement without traceable support used in manual assertion

F

Seeded From UniProt

complete

part_of

GO:0002169

3-methylcrotonyl-CoA carboxylase complex, mitochondrial

PMID:11401427[5]

ECO:0000303

author statement without traceable support used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0005759

mitochondrial matrix

PMID:16023992[6]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

enables

GO:0009374

biotin binding

PMID:11401427[5]

ECO:0000303

author statement without traceable support used in manual assertion

F

Seeded From UniProt

complete

involved_in

GO:0006768

biotin metabolic process

PMID:11401427[5]

ECO:0000303

author statement without traceable support used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0006552

leucine catabolic process

PMID:11170888[2]

ECO:0000303

author statement without traceable support used in manual assertion

P

Seeded From UniProt

complete

part_of

GO:0005759

mitochondrial matrix

PMID:11170888[2]

ECO:0000303

author statement without traceable support used in manual assertion

C

Seeded From UniProt

complete

contributes_to

GO:0004485

methylcrotonoyl-CoA carboxylase activity

PMID:17360195[1]

ECO:0000314

direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000052

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

enables

GO:0005524

ATP binding

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR005479
InterPro:IPR011761
InterPro:IPR013815

F

Seeded From UniProt

complete

enables

GO:0046872

metal ion binding

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR011761

F

Seeded From UniProt

complete

enables

GO:0004485

methylcrotonoyl-CoA carboxylase activity

GO_REF:0000003

ECO:0000501

evidence used in automatic assertion

EC:6.4.1.4

F

Seeded From UniProt

complete

part_of

GO:0002169

3-methylcrotonyl-CoA carboxylase complex, mitochondrial

PMID:22869039[4]

ECO:0000304

author statement supported by traceable reference used in manual assertion

C

Seeded From UniProt

complete

involved_in

GO:0009083

branched-chain amino acid catabolic process

Reactome:R-HSA-70895

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0006768

biotin metabolic process

Reactome:R-HSA-196780

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

part_of

GO:0005829

cytosol

Reactome:R-HSA-9035987
Reactome:R-HSA-3323111
Reactome:R-HSA-2993799

ECO:0000304

author statement supported by traceable reference used in manual assertion



C

Seeded From UniProt

complete

part_of

GO:0005759

mitochondrial matrix

Reactome:R-HSA-70773
Reactome:R-HSA-508308
Reactome:R-HSA-3323111
Reactome:R-HSA-3065959

ECO:0000304

author statement supported by traceable reference used in manual assertion




C

Seeded From UniProt

complete

enables

GO:0000166

nucleotide binding

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0547

F

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0496

C

Seeded From UniProt

complete

enables

GO:0016874

ligase activity

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0436

F

Seeded From UniProt

complete

enables

GO:0005524

ATP binding

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0067

F

Seeded From UniProt

complete

part_of

GO:0005759

mitochondrial matrix

GO_REF:0000039

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-SubCell:SL-0170

C

Seeded From UniProt

complete

involved_in

GO:0006552

leucine catabolic process

GO_REF:0000041

ECO:0000322

imported manually asserted information used in automatic assertion

UniPathway:UPA00363

P

Seeded From UniProt

complete

edit table

Notes

References

See Help:References for how to manage references in GONUTS.

  1. ↑ 1.0 1.1 1.2 Chu, CH & Cheng, D (2007) Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC). Protein Expr. Purif. 53 421-7 PubMed GONUTS page
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 Gallardo, ME et al. (2001) The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Am. J. Hum. Genet. 68 334-46 PubMed GONUTS page
  3. ↑ 3.0 3.1 Baumgartner, MR et al. (2001) The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency. J. Clin. Invest. 107 495-504 PubMed GONUTS page
  4. ↑ 4.0 4.1 4.2 Tong, L (2013) Structure and function of biotin-dependent carboxylases. Cell. Mol. Life Sci. 70 863-91 PubMed GONUTS page
  5. ↑ 5.0 5.1 5.2 5.3 Obata, K et al. (2001) Human biotin-containing subunit of 3-methylcrotonyl-CoA carboxylase gene (MCCA): cDNA sequence, genomic organization, localization to chromosomal band 3q27, and expression. Genomics 72 145-52 PubMed GONUTS page
  6. ↑ Stadler, SC et al. (2005) Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase. Biochem. Biophys. Res. Commun. 334 939-46 PubMed GONUTS page
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