Gene/Proteome Database (LMPD)

LMPD ID
LMP013124
Gene ID
294972
Species
Rattus norvegicus (Rat)
Gene Name
methylcrotonoyl-CoA carboxylase 1 (alpha)
Gene Symbol
Mccc1
Alternate Names
methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; methylcrotonoyl-Coenzyme A carboxylase 1 (alpha); MCCase subunit alpha {ECO:0000250|UniProtKB:Q96RQ3}; mccc1 {ECO:0000312|EMBL:AAH88473.1, ECO:0000312|RGD:1310615}; 3-methylcrotonyl-CoA carboxylase 1 {ECO:0000250|UniProtKB:Q96RQ3}; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha {ECO:0000250|UniProtKB:Q96RQ3}; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit {ECO:0000250|UniProtKB:Q96RQ3}; methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial {ECO:0000250|UniProtKB:Q96RQ3};
Chromosome
2
Map Location
2q25
EC Number
6.4.1.4

Proteins

methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Refseq ID NP_001009653
Protein GI 57528264
UniProt ID Q5I0C3
mRNA ID NM_001009653
Length 715
MAAAALLAAVDRNQLRRVPILLLQPREWPWKHRTVKYGTTPGGSITKVLIANRGEIACRVIRTARKMGVQSVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLAMEKIIQVAKSSAAQAIHPGYGFLSENMEFAEFCKQEGIIFIGPPSTAIRDMGIKSTSKSIMAAAGVPVVEGYHGNDQSDECLKEHAGKIGYPVMIKAIRGGGGKGMRIIRSEKEFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIDPEVRRRLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFYFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEIPLQGHAFEARIYAEDPDNNFMPGAGPLVHLSTPPPDMSTRIETGVRQGDEVSVHYDPMIAKLVVWASDRQSALSKLRYSLHQYNIVGLRTNVDFLLRLSGHSEFEAGNVHTDFIPQHHKDLLPTHSTIAKESVCQAALGLILKEKEMTSAFKLHTQDQFSPFSFSSGRRLNISYTRNMTLRSGKNDIIIAVTYNRDGSYDMQIENKLFRVLGDLSNEDGYTYLKSSVNGVASKSKFILLDNTIYLFSMEGSIEVGIPVPKYLSPVSAEGTQGGTIAPMTGTIEKVFVKAGDRVKAGDALMVMIAMKMEHTIKAPKDGRIKKVFFSEGAQANRHAPLVEFEEEEV
transit_peptide: 1..38 experiment: experimental evidence, no additional details recorded note: Mitochondrion. {ECO:0000250}; propagated from UniProtKB/Swiss-Prot (Q5I0C3.1) calculated_mol_wt: 4439 peptide sequence: MAAAALLAAVDRNQLRRVPILLLQPREWPWKHRTVKYG mat_peptide: 39..715 product: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q5I0C3.1) calculated_mol_wt: 74909 peptide sequence: TTPGGSITKVLIANRGEIACRVIRTARKMGVQSVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLAMEKIIQVAKSSAAQAIHPGYGFLSENMEFAEFCKQEGIIFIGPPSTAIRDMGIKSTSKSIMAAAGVPVVEGYHGNDQSDECLKEHAGKIGYPVMIKAIRGGGGKGMRIIRSEKEFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIDPEVRRRLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFYFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEIPLQGHAFEARIYAEDPDNNFMPGAGPLVHLSTPPPDMSTRIETGVRQGDEVSVHYDPMIAKLVVWASDRQSALSKLRYSLHQYNIVGLRTNVDFLLRLSGHSEFEAGNVHTDFIPQHHKDLLPTHSTIAKESVCQAALGLILKEKEMTSAFKLHTQDQFSPFSFSSGRRLNISYTRNMTLRSGKNDIIIAVTYNRDGSYDMQIENKLFRVLGDLSNEDGYTYLKSSVNGVASKSKFILLDNTIYLFSMEGSIEVGIPVPKYLSPVSAEGTQGGTIAPMTGTIEKVFVKAGDRVKAGDALMVMIAMKMEHTIKAPKDGRIKKVFFSEGAQANRHAPLVEFEEEEV

Gene Information

Entrez Gene ID
294972
Gene Name
methylcrotonoyl-CoA carboxylase 1 (alpha)
Gene Symbol
Mccc1
Species
Rattus norvegicus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005739 IEA:UniProtKB-KW 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

KEGG Pathway Links

KEGG Pathway ID Description
M00036 Leucine degradation, leucine => acetoacetate + acetyl-CoA
rno01100 Metabolic pathways
ko00280 Valine, leucine and isoleucine degradation
rno00280 Valine, leucine and isoleucine degradation

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-CoA carboxylase 1 (alpha)
Protein Entry
MCCA_RAT
UniProt ID
Q5I0C3
Species
Rat

Comments

Comment Type Description
Catalytic Activity ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA
Cofactor Name=biotin; Xref=ChEBI:CHEBI:57586; Evidence= ; 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
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
Similarity Contains 1 biotinyl-binding domain
Subcellular Location Mitochondrion matrix .
Subunit Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits

Identical and Related Proteins

Unique RefSeq proteins for LMP013124 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
57528264 RefSeq NP_001009653 715 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Identical Sequences to LMP013124 proteins

Reference Database Accession Length Protein Name
GI:57528264 GenBank AAH88473.1 715 Methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) [Rattus norvegicus]
GI:57528264 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]

Related Sequences to LMP013124 proteins

Reference Database Accession Length Protein Name
GI:57528264 GenBank AAH21382.1 717 Methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) [Mus musculus]
GI:57528264 GenBank AAH88473.1 715 Methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) [Rattus norvegicus]
GI:57528264 RefSeq NP_076133.3 717 methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [Mus musculus]
GI:57528264 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]