Methylcrotonyl-CoA carboxylase

methylcrotonoyl-CoA carboxylase
Identifiers
Aliasesmethylcrotonyl-CoA carboxylase3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)beta-methylcrotonyl coenzyme A carboxylaseMCCCmethylcrotonyl coenzyme A carboxylasebeta-methylcrotonyl CoA carboxylasebeta-methylcrotonyl-CoA carboxylase
External IDsGeneCards: [1]; OMA:- orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

n/a

n/a

RefSeq (protein)

n/a

n/a

Location (UCSC)n/an/a
PubMed searchn/an/a
Wikidata
View/Edit Human
Methylcrotonoyl-coenzyme A carboxylase 1 (alpha)
Identifiers
SymbolMCCC1
NCBI gene56922
HGNC6936
OMIM609010
RefSeqNM_020166
UniProtQ96RQ3
Other data
EC number6.4.1.4
LocusChr. 3 q27.1
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StructuresSwiss-model
DomainsInterPro
Methylcrotonoyl-coenzyme A carboxylase 2 (beta)
Identifiers
SymbolMCCC2
NCBI gene64087
HGNC6937
OMIM609014
RefSeqNM_022132
UniProtQ9HCC0
Other data
EC number6.4.1.4
LocusChr. 5 q12-q13
Search for
StructuresSwiss-model
DomainsInterPro

Methylcrotonyl CoA carboxylase (EC 6.4.1.4, MCC) (3-methylcrotonyl CoA carboxylase, methylcrotonoyl-CoA carboxylase) is a biotin-requiring enzyme located in the mitochondria. MCC uses bicarbonate as a carboxyl group source to catalyze the carboxylation of a carbon adjacent to a carbonyl group performing the fourth step in processing leucine, an essential amino acid.[1]

  1. ^ Bruice PY (2001). Organic chemistry: study guide and solutions manual (2nd ed.). Upper Saddle River, N.J.: Prentice Hall. pp. 1010–11. ISBN 978-0-13-017859-6.