Gene/Proteome Database (LMPD)

LMPD ID
LMP013461
Gene ID
361884
Species
Rattus norvegicus (Rat)
Gene Name
methylcrotonoyl-CoA carboxylase 2 (beta)
Gene Symbol
Mccc2
Alternate Names
methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; MCCase subunit beta; 3-methylcrotonyl-CoA carboxylase 2; methylcrotonoyl-Coenzyme A carboxylase 2 (beta); 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit;
Chromosome
2
Map Location
2q12
EC Number
6.4.1.4

Proteins

methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
Refseq ID NP_001012177
Protein GI 58865926
UniProt ID Q5XIT9
mRNA ID NM_001012177
Length 563
MWGALRSVLRPCSRASVPRQRAYHGDAVARLGTQPDSGSSTYQENYEQMKALVNQLHERAQYVRLGGSEKARARHTSRGKLLPRDRIDNLIDPGSPFLEFSQFAGYKLYGEEEVPAGGIITGIGRVSGVECMIVANDATVKGGTYYPVTVKKHVRAQEIALQNRLPCIYLVDSGGANLPRQADTFPDRDHFGRIFYNQAIMSSKNITQIAVVMGSCTAGGAYVPAMADENIIVQRQGTIFLAGPPLVKAATGEEVSAEDLGGADLHCRRSGVTDHYALDDHHALHLTRKVVRSLNYQKKLDVTVEPSEEPLFPADELYGIVGANLKRSFDVREVIARIVDGSRFNEFKALYGDTLVTGFARIFGYPVGIIGNNGVLFSESAKKGAHFVQLCCQRNIPLLFLQNITGFMVGKDYEAEGIAKDGAKMVAAVSCAKVPKITVIIGGSYGAGNYGMCGRAYSPRFLYMWPNARISVMGGEQAATVLATVARDQRAREGKQFSSAEEAALKEPIIKRFEEEGNPYYSSARLWDDGIIDPVDTRLVLGLSISAALNAPIQRTDFGIFRM
transit_peptide: 1..22 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (Q5XIT9.1) calculated_mol_wt: 2498 peptide sequence: MWGALRSVLRPCSRASVPRQRA mat_peptide: 23..563 product: Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q5XIT9.1) calculated_mol_wt: 59038 peptide sequence: YHGDAVARLGTQPDSGSSTYQENYEQMKALVNQLHERAQYVRLGGSEKARARHTSRGKLLPRDRIDNLIDPGSPFLEFSQFAGYKLYGEEEVPAGGIITGIGRVSGVECMIVANDATVKGGTYYPVTVKKHVRAQEIALQNRLPCIYLVDSGGANLPRQADTFPDRDHFGRIFYNQAIMSSKNITQIAVVMGSCTAGGAYVPAMADENIIVQRQGTIFLAGPPLVKAATGEEVSAEDLGGADLHCRRSGVTDHYALDDHHALHLTRKVVRSLNYQKKLDVTVEPSEEPLFPADELYGIVGANLKRSFDVREVIARIVDGSRFNEFKALYGDTLVTGFARIFGYPVGIIGNNGVLFSESAKKGAHFVQLCCQRNIPLLFLQNITGFMVGKDYEAEGIAKDGAKMVAAVSCAKVPKITVIIGGSYGAGNYGMCGRAYSPRFLYMWPNARISVMGGEQAATVLATVARDQRAREGKQFSSAEEAALKEPIIKRFEEEGNPYYSSARLWDDGIIDPVDTRLVLGLSISAALNAPIQRTDFGIFRM

Gene Information

Entrez Gene ID
361884
Gene Name
methylcrotonoyl-CoA carboxylase 2 (beta)
Gene Symbol
Mccc2
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:0004485 ISS:UniProtKB F methylcrotonoyl-CoA carboxylase activity
GO:0015936 IDA:RGD P coenzyme A metabolic process
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

REACTOME Pathway Links

REACTOME Pathway ID Description
5954011 Biotin transport and metabolism
5953282 Branched-chain amino acid catabolism
5953298 Defective AMN causes hereditary megaloblastic anemia 1
5953308 Defective BTD causes biotidinase deficiency
5953307 Defective CD320 causes methylmalonic aciduria
5953299 Defective CUBN causes hereditary megaloblastic anemia 1
5953296 Defective GIF causes intrinsic factor deficiency
5953310 Defective HLCS causes multiple carboxylase deficiency
5953297 Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF
5953305 Defective MMAA causes methylmalonic aciduria type cblA
5953302 Defective MMAB causes methylmalonic aciduria type cblB
5953304 Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC
5953303 Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD
5953301 Defective MTR causes methylmalonic aciduria and homocystinuria type cblG
5953300 Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE
5953306 Defective MUT causes methylmalonic aciduria mut type
5953293 Defective TCN2 causes hereditary megaloblastic anemia
5953309 Defects in biotin (Btn) metabolism
5953294 Defects in cobalamin (B12) metabolism
5953295 Defects in vitamin and cofactor metabolism
5953253 Disease
5953250 Metabolism
5953272 Metabolism of amino acids and derivatives
5953292 Metabolism of vitamins and cofactors
5953291 Metabolism of water-soluble vitamins and cofactors

Domain Information

InterPro Annotations

Accession Description
IPR011763 Acetyl-coenzyme A carboxyltransferase, C-terminal
IPR011762 Acetyl-coenzyme A carboxyltransferase, N-terminal
IPR000022 Carboxyl transferase
IPR029045 ClpP/crotonase-like domain

UniProt Annotations

Entry Information

Gene Name
methylcrotonoyl-CoA carboxylase 2 (beta)
Protein Entry
MCCB_RAT
UniProt ID
Q5XIT9
Species
Rat

Comments

Comment Type Description
Catalytic Activity ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.
Function Carboxyltransferase 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 Belongs to the AccD/PCCB family
Similarity Contains 1 carboxyltransferase 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 LMP013461 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
58865926 RefSeq NP_001012177 563 methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

Identical Sequences to LMP013461 proteins

Reference Database Accession Length Protein Name
GI:58865926 GenBank AAH83581.1 563 Methylcrotonoyl-Coenzyme A carboxylase 2 (beta) [Rattus norvegicus]
GI:58865926 SwissProt Q5XIT9.1 563 RecName: Full=Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; Short=MCCase subunit beta; AltName: Full=3-methylcrotonyl-CoA carboxylase 2; AltName: Full=3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; AltName: Full=3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta; Flags: Precursor [Rattus norvegicus]

Related Sequences to LMP013461 proteins

Reference Database Accession Length Protein Name
GI:58865926 DBBJ BAE27475.1 563 unnamed protein product [Mus musculus]
GI:58865926 DBBJ BAE27489.1 563 unnamed protein product [Mus musculus]
GI:58865926 GenBank AAH83581.1 563 Methylcrotonoyl-Coenzyme A carboxylase 2 (beta) [Rattus norvegicus]
GI:58865926 SwissProt Q5XIT9.1 563 RecName: Full=Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; Short=MCCase subunit beta; AltName: Full=3-methylcrotonyl-CoA carboxylase 2; AltName: Full=3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; AltName: Full=3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta; Flags: Precursor [Rattus norvegicus]