Gene/Proteome Database (LMPD)

LMPD ID
LMP003837
Gene ID
56922
Species
Homo sapiens (Human)
Gene Name
methylcrotonoyl-CoA carboxylase 1 (alpha)
Gene Symbol
MCCC1
Synonyms
MCC-B; MCCA
Chromosome
3
Map Location
3q27
Summary
This gene encodes the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for MCCC1

Proteins

methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 precursor
Refseq ID NP_064551
Protein GI 116805327
UniProt ID Q96RQ3
mRNA ID NM_020166
Length 725
RefSeq Status VALIDATED
MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNITKVLIANRGEIACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLSMEKIIQVAKTSAAQAIHPGCGFLSENMEFAELCKQEGIIFIGPPPSAIRDMGIKSTSKSIMAAAGVPVVEGYHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRKKLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLHTNIDFLLNLSGHPEFEAGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSSSGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKCSVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESDKRESE
transit_peptide: 1..41 experiment: experimental evidence, no additional details recorded note: Mitochondrion; propagated from UniProtKB/Swiss-Prot (Q96RQ3.3) calculated_mol_wt: 4833 peptide sequence: MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKY mat_peptide: 42..725 product: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q96RQ3.3) calculated_mol_wt: 75659 peptide sequence: TTATGRNITKVLIANRGEIACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLSMEKIIQVAKTSAAQAIHPGCGFLSENMEFAELCKQEGIIFIGPPPSAIRDMGIKSTSKSIMAAAGVPVVEGYHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRKKLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLHTNIDFLLNLSGHPEFEAGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSSSGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKCSVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESDKRESE
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 2
Refseq ID NP_001280202
Protein GI 647569552
UniProt ID Q68D27
mRNA ID NM_001293273
Length 608
RefSeq Status VALIDATED
MGVEAKNHEVHNSHRQMKHIPSAPLPPSRATYLWRKSFKWPRPLLHSTSKSIMAAAGVPVVEGYHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRKKLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLHTNIDFLLNLSGHPEFEAGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSSSGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKCSVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESDKRESE

Gene Information

Entrez Gene ID
56922
Gene Name
methylcrotonoyl-CoA carboxylase 1 (alpha)
Gene Symbol
MCCC1
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005524 IEA:InterPro F ATP binding
GO:0004075 IEA:InterPro F biotin carboxylase activity
GO:0046872 IEA:InterPro F metal ion binding

BIOCYC Pathway Links

BIOCYC Pathway ID Description
LEU-DEG2-PWY leucine degradation I

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_11153 Biotin transport and metabolism
REACT_197 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
IPR005482 Biotin carboxylase, C-terminal
IPR011764 Biotin carboxylation domain
IPR001882 Biotin-binding site
IPR000089 Biotin/lipoyl attachment
IPR005479 Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain
IPR011054 Rudiment single hybrid motif
IPR011053 Single hybrid motif

UniProt Annotations

Entry Information

Gene Name
methylcrotonoyl-CoA carboxylase 1 (alpha)
Protein Entry
MCCA_HUMAN
UniProt ID
Q96RQ3
Species
Human

Identical and Related Proteins

Unique RefSeq proteins for LMP003837 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
116805327 RefSeq NP_064551 725 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 precursor
647569552 RefSeq NP_001280202 608 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 2

Identical Sequences to LMP003837 proteins

Reference Database Accession Length Protein Name
GI:116805327 DBBJ BAA99407.1 725 3-methylcrotonyl-CoA carboxylase biotin-containing subunit [Homo sapiens]
GI:116805327 GenBank AAG53095.1 725 3-methylcrotonyl-CoA carboxylase alpha subunit [Homo sapiens]
GI:116805327 GenBank AED35469.1 725 Sequence 128 from patent US 7879989
GI:116805327 GenBank AED35475.1 725 Sequence 135 from patent US 7879989
GI:116805327 GenBank AFX58141.1 725 Sequence 94 from patent US 8309323
GI:116805327 SwissProt Q96RQ3.3 725 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 [Homo sapiens]

Related Sequences to LMP003837 proteins

Reference Database Accession Length Protein Name
GI:116805327 EMBL CBF64321.1 725 unnamed protein product [Homo sapiens]
GI:116805327 GenBank AGX52995.1 725 Sequence 6341 from patent US 8541208
GI:116805327 GenBank AGX53931.1 725 Sequence 8212 from patent US 8541208
GI:116805327 GenBank AGX56198.1 725 Sequence 12722 from patent US 8541208
GI:116805327 GenBank AGX60613.1 725 Sequence 21457 from patent US 8541208
GI:116805327 GenBank AGX77246.1 725 Sequence 54266 from patent US 8541208
GI:647569552 RefSeq XP_007970143.1 669 PREDICTED: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X4 [Chlorocebus sabaeus]
GI:647569552 RefSeq XP_007970147.1 613 PREDICTED: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X8 [Chlorocebus sabaeus]
GI:647569552 RefSeq XP_008955612.1 608 PREDICTED: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X3 [Pan paniscus]
GI:647569552 RefSeq XP_009199552.1 613 PREDICTED: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X4 [Papio anubis]
GI:647569552 RefSeq XP_009199555.1 608 PREDICTED: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X5 [Papio anubis]
GI:647569552 RefSeq XP_009445188.1 608 PREDICTED: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X3 [Pan troglodytes]