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Methylmalonic acidemia without homocystinuria

ORPHA:293355

Definition

Methylmalonic acidemia is an inborn error of vitamin B12 metabolism characterized by gastrointestinal and neurometabolic manifestations resulting from decreased function of the mitochondrial enzyme methylmalonyl-CoA mutase.

Prevalence
1-9 / 100 000
Inheritance
Autosomal dominant, Autosomal recessive, X-linked dominant
Age of onset
All ages