Methods are disclosed for treating a subject with a cobalamin C (cblC) deficiency. These methods include selecting a human subject with the cblC deficiency; and administering about 5 mg to about 10 grams of methylcobalamin (MeCbl) daily to the human subject. Methods also are disclosed for treating a fetus with a cobalamin C (cblC) deficiency. The methods include selecting a female human subject pregnant with the fetus that has the cblC deficiency; and administering about 5 mg to about 10 grams of MeCbl daily to the female human subject, in order to treat the cblC deficiency in the fetus. Optionally, OHCbl can be administered to the subject.
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