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3-methylcrotonyl-CoA carboxylase-alpha (3-methylcrotonyl-CoA:carbon dioxide ligase alpha subunit, 3-methylcrotonyl-CoA carboxylase 1, MCCase subunit alpha, 3- methylcrotonyl-CoA carboxylase biotin-containing subunit, MCCC1, MCCA)

Function: - 3-methylcrotonyl-CoA carboxylase alpha subunit - leucine catabolism Cofactor: biotin Structure: - contains 1 ATP-grasp domain - contains 1 biotin carboxylation domain - contains 1 biotinyl-binding domain Compartment: mitochondrial matrix Pathology: - defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 1

General

mitochondrial protein protein subunit

Properties

SIZE: entity length = 725 aa MW = 80 kD COMPARTMENT: mitochondria MOTIF: Biotin carboxylation {48-494} MOTIF: ATP-binding site NAME: ATP-binding site SITE: 163-163 ATP-grasp domain SITE: 167-364 ATP-binding site NAME: ATP-binding site SITE: 247-247 ATP-binding site NAME: ATP-binding site SITE: 282-282 arginine residue {R339} binding site SITE: 629-714 FOR-BINDING-OF: biotin MOTIF: binding site SITE: 681-681 FOR-BINDING-OF: biotin

Database Correlations

OMIM 210200 UniProt Q96RQ3 PFAM correlations Kegg hsa:5692 ENZYME EC_6.4.1.4

References

  1. UniProt :accession Q96RQ3
  2. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=MCCC1

Component-of

3-methylcrotonyl-CoA carboxylase (MCCase, 3-methylcrotonyl-CoA:carbon dioxide ligase)