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HUMAN:MCCB

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Species (Taxon ID) Homo sapiens (Human). (9606)
Gene Name(s) MCCC2 (synonyms: MCCB)
Protein Name(s) Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

MCCase subunit beta 3-methylcrotonyl-CoA carboxylase 2 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta

External Links
UniProt Q9HCC0
EMBL AB050049
AB050050
AF310971
AF301000
AF261884
AC138832
CH471084
BC014897
BC065027
AL079298
CCDS CCDS34184.1
RefSeq NP_071415.1
XP_005248624.1
UniGene Hs.604789
ProteinModelPortal Q9HCC0
SMR Q9HCC0
BioGrid 122050
IntAct Q9HCC0
MINT MINT-8051924
STRING 9606.ENSP00000343657
DrugBank DB00121
PhosphoSite Q9HCC0
DMDM 20138731
REPRODUCTION-2DPAGE IPI00784044
MaxQB Q9HCC0
PaxDb Q9HCC0
PRIDE Q9HCC0
Ensembl ENST00000340941
GeneID 64087
KEGG hsa:64087
UCSC uc003kbs.4
CTD 64087
GeneCards GC05P070883
HGNC HGNC:6937
HPA HPA038300
HPA038301
MIM 210210
609014
neXtProt NX_Q9HCC0
Orphanet 6
PharmGKB PA30681
eggNOG COG4799
GeneTree ENSGT00530000063337
HOGENOM HOG000218692
HOVERGEN HBG052424
InParanoid Q9HCC0
KO K01969
OMA CKTSGVT
PhylomeDB Q9HCC0
TreeFam TF300446
BioCyc MetaCyc:ENSG00000131844-MONOMER
Reactome REACT_11153
REACT_169312
REACT_197
UniPathway UPA00363
GenomeRNAi 64087
NextBio 65884
PRO PR:Q9HCC0
Proteomes UP000005640
Bgee Q9HCC0
CleanEx HS_MCCC2
ExpressionAtlas Q9HCC0
Genevestigator Q9HCC0
GO GO:0005829
GO:0005759
GO:0005739
GO:0005524
GO:0004485
GO:0006768
GO:0009083
GO:0034641
GO:0015936
GO:0006552
GO:0044281
GO:0006766
GO:0006767
Gene3D 3.90.226.10
InterPro IPR000022
IPR029045
IPR011763
IPR011762
Pfam PF01039
SUPFAM SSF52096
PROSITE PS50989
PS50980

Annotations

Qualifier GO ID GO term name Reference ECO ID ECO term name with/from Aspect Extension Notes Status
GO:0004485

3-methylcrotonyl-CoA carboxylase complex, mitochondrial

PMID:17360195[1]

ECO:0000314

C

Figure 3

complete
CACAO 5314

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

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

part_of

GO:0005759

mitochondrial matrix

PMID:16023992[4]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

PMID:11170888[2]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

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:17360195[1]

ECO:0000314

direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

part_of

GO:1905202

methylcrotonoyl-CoA carboxylase complex

PMID:21873635[5]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

PANTHER:PTN000523406
UniProtKB:Q9HCC0
UniProtKB:Q9I297

C

Seeded From UniProt

complete

involved_in

GO:0006552

leucine catabolic process

PMID:21873635[5]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

PANTHER:PTN000523406
TAIR:locus:2124256
UniProtKB:C8VAZ1

P

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

PMID:21873635[5]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

PANTHER:PTN000523407
TAIR:locus:2124256
UniProtKB:Q9HCC0

C

Seeded From UniProt

complete

contributes_to

GO:0004485

methylcrotonoyl-CoA carboxylase activity

PMID:21873635[5]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

PANTHER:PTN000523406
RGD:1310279
UniProtKB:C8VAZ1
UniProtKB:Q9HCC0
UniProtKB:Q9I297

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

involved_in

GO:0015936

coenzyme A metabolic process

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q5XIT9
ensembl:ENSRNOP00000023900

P

Seeded From UniProt

complete

enables

GO:0004485

methylcrotonoyl-CoA carboxylase activity

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q5XIT9
ensembl:ENSRNOP00000023900

F

Seeded From UniProt

complete

enables

GO:0016874

ligase activity

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR011763

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[6]

ECO:0000304

author statement supported by traceable reference used in manual assertion

C

Seeded From UniProt

complete

involved_in

GO:0006552

leucine catabolic process

PMID:11170888[2]

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

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

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: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

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 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 3.2 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. ↑ 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
  5. ↑ 5.0 5.1 5.2 5.3 Gaudet, P et al. (2011) Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium. Brief. Bioinformatics 12 449-62 PubMed GONUTS page
  6. ↑ Tong, L (2013) Structure and function of biotin-dependent carboxylases. Cell. Mol. Life Sci. 70 863-91 PubMed GONUTS page
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