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Here, the enzyme catalyzes the reversible isomerization of l-methylmalonyl-CoA to succinyl-CoA using AdoCbl as a cofactor

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Of the three haplotypes, the upstream genetic alterations that were in linkage disequilibrium with the Leu allele were also found to have the lowest level of activity in a transcription reporter gene assay, causing a 25% suppression in promoter activity (152)