Gene/Proteome Database (LMPD)

LMPD ID
LMP003171
Gene ID
64087
Species
Homo sapiens (Human)
Gene Name
methylcrotonoyl-CoA carboxylase 2 (beta)
Gene Symbol
MCCC2
Synonyms
MCCB
Alternate Names
methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; biotin carboxylase; 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; non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase
Chromosome
5
Map Location
5q12-q13
EC Number
6.4.1.4
Summary
This gene encodes the small 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 MCCC2

Proteins

methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
Refseq ID NP_071415
Protein GI 11545863
UniProt ID Q9HCC0
mRNA ID NM_022132
Length 563
RefSeq Status REVIEWED
MWAVLRLALRPCARASPAGPRAYHGDSVASLGTQPDLGSALYQENYKQMKALVNQLHERVEHIKLGGGEKARALHISRGKLLPRERIDNLIDPGSPFLELSQFAGYQLYDNEEVPGGGIITGIGRVSGVECMIIANDATVKGGAYYPVTVKKQLRAQEIAMQNRLPCIYLVDSGGAYLPRQADVFPDRDHFGRTFYNQAIMSSKNIAQIAVVMGSCTAGGAYVPAMADENIIVRKQGTIFLAGPPLVKAATGEEVSAEDLGGADLHCRKSGVSDHWALDDHHALHLTRKVVRNLNYQKKLDVTIEPSEEPLFPADELYGIVGANLKRSFDVREVIARIVDGSRFTEFKAFYGDTLVTGFARIFGYPVGIVGNNGVLFSESAKKGTHFVQLCCQRNIPLLFLQNITGFMVGREYEAEGIAKDGAKMVAAVACAQVPKITLIIGGSYGAGNYGMCGRAYSPRFLYIWPNARISVMGGEQAANVLATITKDQRAREGKQFSSADEAALKEPIIKKFEEEGNPYYSSARVWDDGIIDPADTRLVLGLSFSAALNAPIEKTDFGIFRM

Gene Information

Entrez Gene ID
64087
Gene Name
methylcrotonoyl-CoA carboxylase 2 (beta)
Gene Symbol
MCCC2
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005829 TAS:Reactome C cytosol
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005739 NAS:UniProtKB C mitochondrion
GO:0005524 IEA:UniProtKB-KW F ATP binding
GO:0004485 IDA:UniProtKB F methylcrotonoyl-CoA carboxylase activity
GO:0006768 TAS:Reactome P biotin metabolic process
GO:0009083 TAS:Reactome P branched-chain amino acid catabolic process
GO:0034641 TAS:Reactome P cellular nitrogen compound metabolic process
GO:0015936 IEA:Ensembl P coenzyme A metabolic process
GO:0006552 TAS:UniProtKB P leucine catabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0006766 TAS:Reactome P vitamin metabolic process
GO:0006767 TAS:Reactome P water-soluble vitamin metabolic process

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
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_HUMAN
UniProt ID
Q9HCC0
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9HCC0-1; Sequence=Displayed; Name=2; IsoId=Q9HCC0-2; Sequence=VSP_000069; Note=No experimental confirmation available.;
Biophysicochemical Properties Kinetic parameters: KM=45 uM for ATP ; KM=74 uM for 3-methylcrotonyl-CoA ; Note=kcat is 4.0 sec(-1).;
Catalytic Activity ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.
Disease Methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D) [MIM
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
Sequence Caution Sequence=AAH14897.1; Type=Frameshift; Positions=359; Evidence= ;
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 LMP003171 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
11545863 RefSeq NP_071415 563 methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

Identical Sequences to LMP003171 proteins

Reference Database Accession Length Protein Name
GI:11545863 GenBank ADT50540.1 563 Sequence 2557 from patent US 7842467
GI:11545863 GenBank AED35476.1 563 Sequence 136 from patent US 7879989
GI:11545863 GenBank AEF64009.1 563 Sequence 305 from patent US 7932031
GI:11545863 GenBank AEF64056.1 563 Sequence 352 from patent US 7932031
GI:11545863 GenBank AFX58142.1 563 Sequence 96 from patent US 8309323
GI:11545863 GenBank AIC56982.1 563 MCCC2, partial [synthetic construct]

Related Sequences to LMP003171 proteins

Reference Database Accession Length Protein Name
GI:11545863 GenBank JAA04344.1 563 methylcrotonoyl-CoA carboxylase 2 (beta) [Pan troglodytes]
GI:11545863 GenBank JAA17982.1 563 methylcrotonoyl-CoA carboxylase 2 (beta) [Pan troglodytes]
GI:11545863 GenBank JAA29128.1 563 methylcrotonoyl-CoA carboxylase 2 (beta) [Pan troglodytes]
GI:11545863 GenBank JAA35437.1 563 methylcrotonoyl-CoA carboxylase 2 (beta) [Pan troglodytes]
GI:11545863 GenBank JAA35438.1 563 methylcrotonoyl-CoA carboxylase 2 (beta) [Pan troglodytes]
GI:11545863 RefSeq XP_003814024.1 563 PREDICTED: methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform X1 [Pan paniscus]