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1. Vitamin B12 is essential for the function of two human enzymes, methionine synthase and methylmalonyl-CoA mutase (MUT), which require different forms of cobalamin as cofactors.

2. MMAA, a processing protein involved in the mitochondrial assembly of AdoCbl into MUT, interacts with MUT and modulates its GTPase activity.

3. The interaction between MMAA and MUT is nucleotide-selective for MMAA and apoenzyme-dependent for MUT, and plays a gatekeeping role in AdoCbl assembly. A homoallelic patient mutation of MMAA, G188R, abrogates this interaction.

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