disease has basis in dysfunction of MMUT methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency DOID:0060740 GARD:0003586 ICD10:E71.1 MCM deficiency MONDO:0009612 NCIT:C148366 OMIM:251000 Orphanet:27 UMLS:C1855114 Vitamin B12-unresponsive methylmalonic acidemia is an inborn error of vitamin B12 (cobalamin) metabolism characterized by recurrent ketoacidotic crises or transient vomiting, dehydration, hypotonia and intellectual deficit, which does not respond to administration of vitamin B12. There are two types of vitamin B12-unresponsive methylmalonic acidemia: mut0 and mut-. methylmalonic acidemia due to methylmalonyl-CoA mutase deficiency methylmalonic acidemia due to methylmalonyl-Coa mutase deficiency MMA due to MCM deficiency methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency methylmalonic aciduria mut type methylmalonic aciduria, mut type methylmalonic aciduria, mut(-) type methylmalonic aciduria, mut(0) type methylmalonyl-CoA mutase deficiency methylmalonyl-Coenzyme A mutase deficiency vitamin B12-unresponsive methylmalonic acidemia vitamin B12-unresponsive methylmalonic aciduria methylmalonic acidemia without homocystinuria nephropathy secondary to a storage or other metabolic disease