Gene/Proteome Database (LMPD)

LMPD ID
LMP003840
Gene ID
72039
Species
Mus musculus (Mouse)
Gene Name
methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
Gene Symbol
Mccc1
Synonyms
1810045E08Rik; 2310058B18Rik; MCCalpha; Mcca; R75106
Alternate Names
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; MCCase subunit alpha; 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit
Chromosome
3
Map Location
3 B|3
EC Number
6.4.1.4

Proteins

methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Refseq ID NP_076133
Protein GI 186700620
UniProt ID Q99MR8
mRNA ID NM_023644
Length 717
RefSeq Status VALIDATED
MAAAALLAAVDRNQLRRVPILLLQPREWAWKLRTMKYGTTPGGSITKVLIANRGEIACRVIRTAKKMGVQSVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLAMEKIIQVAKSSAAQAIHPGYGFLSENMEFAELCKQEGIIFIGPPSSAIRDMGIKSTSKSIMAAAGVPVVEGYHGKDQSDQCLREHAGKIGYPVMIKAVRGGGGKGMRIVRSEREFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGINPEVRRKLGEAAVRAAKAVKYVGAGTVEFIMDSRHNFYFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEIPLQGHAFEARIYAEDPDNNFMPGAGPLVHLSTPSADMSTRIETGVRQGDEVSVHYDPMIAKLVVWASDRQSALSKLRYCLHQYNIVGLRSNVDFLLRLSGHPEFEAGNVHTDFIPQHHKDLLPSHSTIAKESVCQAALGLILKEKEMTSAFKLHTQDQFSPFSFSSGRRLNISYTRNMTLRSGKSDIVIAVTYNRDGSYDMQIDNKSFRVLGDLSSEDGCTYLKSSINGVARKSKFILLDNTVHLFSMEGSIEVGIPVPKYLSPVSAEGAQGGTIAPMTGTIEKVFVKAGDRVKAGDSLMVMIAMKMEHTIKAPKDGRIKKVFFSEGAQANRHAPLVEFEEEESDK
transit_peptide: 1..38 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (Q99MR8.2) calculated_mol_wt: 4421 peptide sequence: MAAAALLAAVDRNQLRRVPILLLQPREWAWKLRTMKYG mat_peptide: 39..717 product: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q99MR8.2) calculated_mol_wt: 74941 peptide sequence: TTPGGSITKVLIANRGEIACRVIRTAKKMGVQSVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLAMEKIIQVAKSSAAQAIHPGYGFLSENMEFAELCKQEGIIFIGPPSSAIRDMGIKSTSKSIMAAAGVPVVEGYHGKDQSDQCLREHAGKIGYPVMIKAVRGGGGKGMRIVRSEREFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGINPEVRRKLGEAAVRAAKAVKYVGAGTVEFIMDSRHNFYFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEIPLQGHAFEARIYAEDPDNNFMPGAGPLVHLSTPSADMSTRIETGVRQGDEVSVHYDPMIAKLVVWASDRQSALSKLRYCLHQYNIVGLRSNVDFLLRLSGHPEFEAGNVHTDFIPQHHKDLLPSHSTIAKESVCQAALGLILKEKEMTSAFKLHTQDQFSPFSFSSGRRLNISYTRNMTLRSGKSDIVIAVTYNRDGSYDMQIDNKSFRVLGDLSSEDGCTYLKSSINGVARKSKFILLDNTVHLFSMEGSIEVGIPVPKYLSPVSAEGAQGGTIAPMTGTIEKVFVKAGDRVKAGDSLMVMIAMKMEHTIKAPKDGRIKKVFFSEGAQANRHAPLVEFEEEESDK

Gene Information

Entrez Gene ID
72039
Gene Name
methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
Gene Symbol
Mccc1
Species
Mus musculus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005743 IDA:MGI C mitochondrial inner membrane
GO:0005739 IDA:MGI C mitochondrion
GO:0005524 IEA:UniProtKB-KW F ATP binding
GO:0004075 IEA:InterPro F biotin carboxylase activity
GO:0046872 IEA:InterPro F metal ion binding
GO:0004485 ISS:UniProtKB F methylcrotonoyl-CoA carboxylase activity
GO:0006552 IEA:UniProtKB-UniPathway P leucine catabolic process

REACTOME Pathway Links

REACTOME Pathway ID Description
5893521 Biotin transport and metabolism
5892792 Branched-chain amino acid catabolism

Domain Information

InterPro Annotations

Accession Description
IPR011761 ATP-grasp fold
IPR013815 ATP-grasp fold, subdomain 1
IPR013816 ATP-grasp fold, subdomain 2
IPR001882 Biotin-binding site
IPR005482 Biotin carboxylase, C-terminal
IPR011764 Biotin carboxylation domain
IPR000089 Biotin/lipoyl attachment
IPR005481 Carbamoyl-phosphate synthase, large subunit, N-terminal
IPR005479 Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain
IPR016185 Pre-ATP-grasp domain
IPR011054 Rudiment single hybrid motif
IPR011053 Single hybrid motif

UniProt Annotations

Entry Information

Gene Name
methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
Protein Entry
MCCA_MOUSE
UniProt ID
Q99MR8
Species
Mouse

Comments

Comment Type Description
Catalytic Activity ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.
Cofactor Name=biotin; Xref=ChEBI:CHEBI:57586; Note=Biotin.;
Function Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3- methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. {ECO:0000250}.
Pathway Amino-acid degradation; L-leucine degradation; (S)-3- hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
Similarity Contains 1 ATP-grasp domain. {ECO:0000255|PROSITE- ProRule:PRU00409}.
Similarity Contains 1 biotin carboxylation domain. {ECO:0000305}.
Similarity Contains 1 biotinyl-binding domain. {ECO:0000255|PROSITE-ProRule:PRU01066, ECO:0000305}.
Subcellular Location Mitochondrion matrix {ECO:0000250}.
Subunit Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits. {ECO:0000250}.

Identical and Related Proteins

Unique RefSeq proteins for LMP003840 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
186700620 RefSeq NP_076133 717 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Identical Sequences to LMP003840 proteins

Reference Database Accession Length Protein Name
GI:186700620 DBBJ BAB25253.1 717 unnamed protein product [Mus musculus]
GI:186700620 DBBJ BAC27239.1 717 unnamed protein product [Mus musculus]
GI:186700620 DBBJ BAE40458.1 717 unnamed protein product [Mus musculus]
GI:186700620 SwissProt Q99MR8.2 717 RecName: Full=Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; Short=MCCase subunit alpha; AltName: Full=3-methylcrotonyl-CoA carboxylase 1; AltName: Full=3-methylcrotonyl-CoA carboxylase biotin-containing subunit; AltName: Full=3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; Flags: Precursor [Mus musculus]

Related Sequences to LMP003840 proteins

Reference Database Accession Length Protein Name
GI:186700620 GenBank AAG50244.1 717 3-methylcrotonyl-CoA carboxylase alpha subunit [Mus musculus]
GI:186700620 GenBank AAH21382.1 717 Methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) [Mus musculus]
GI:186700620 GenBank AAH88473.1 715 Methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) [Rattus norvegicus]
GI:186700620 RefSeq NP_001009653.1 715 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [Rattus norvegicus]
GI:186700620 RefSeq XP_007614059.1 717 PREDICTED: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [Cricetulus griseus]
GI:186700620 SwissProt Q5I0C3.1 715 RecName: Full=Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; Short=MCCase subunit alpha; AltName: Full=3-methylcrotonyl-CoA carboxylase 1; AltName: Full=3-methylcrotonyl-CoA carboxylase biotin-containing subunit; AltName: Full=3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; Flags: Precursor [Rattus norvegicus]