Structures of the human GTPase MMAA and vitamin B12-dependent methylmalonyl-CoA mutase and insight into their complex formation.

Journal article

Vitamin B(12) (cobalamin, Cbl) is essential to the function of two human enzymes, methionine synthase (MS) and methylmalonyl-CoA mutase (MUT). The conversion of dietary Cbl to its cofactor forms, methyl-Cbl (MeCbl) for MS and adenosyl-Cbl (AdoCbl) for MUT, located in the cytosol and mitochondria, respectively, requires a complex pathway of intracellular processing and trafficking. One of the processing proteins, MMAA (methylmalonic aciduria type A), is implicated in the mitochondrial assembly...

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Authors: Froese, D; Kochan, G; Muniz, JR; Wu, X; Gileadi, C

• Publication status: Published
• Journal: Journal of biological chemistry
• Volume: 285
• Issue: 49
• Pages: 38204-38213
• Publication date: 2010-12-01
• DOI: 10.1074/jbc.m110.177717
• EISSN: 1083-351X
• ISSN: 0021-9258
• Language: English
• Keywords: Cobamides; Mitochondria; Membrane Transport Proteins; Cytosol; Metabolism, Inborn Errors; Humans; Crystallography, X-Ray; Mitochondrial Membrane Transport Proteins; Mutation, Missense; Mitochondrial Proteins; Methylmalonyl-CoA Mutase; Guanosine Triphosphate; Protein Structure, Quaternary; Guanosine Diphosphate; Child; Multiprotein Complexes; Child, Preschool