Conditions / Genetic

methylmalonic acidemia due to transcobalamin receptor defect

info · Genetic · ICD-10: E71.1

A methylmalonic acidemia characterized by autosomal recessive inheritance of low uptake of transcobalamin-bound cobalamin, but normal conversion to adenosylcobalamin and methylcobalamin and that has_material_basis_in mutation in the CD320 gene.

Signs and symptoms

  • Methylmalonic aciduria
  • Methylmalonic acidemia
  • Reduced cellular cobalamin uptake
  • Hyperhomocystinemia

Also known as: methylmalonic acidemia, TCblR type; methylmalonic aciduria due to transcobalamin receptor defect