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A derangement in B12metabolism leading to homocystinemia, cystathioninemia and methylmalonic aciduria

Authors
Journal
Biochemical and Biophysical Research Communications
0006-291X
Publisher
Elsevier
Publication Date
Volume
35
Issue
1
Identifiers
DOI: 10.1016/0006-291x(69)90491-4
Disciplines
  • Biology

Abstract

Abstract In mammals, vitamin B 12 derivatives are known to function as coenzymes for two reactions: (a) methionine formation from 5-methylfolate-H 4 and homocysteine and (b) isomerization of methylmalonyl-CoA to succinyl-CoA (Weissbach and Dickerman,1967; Hogenkamp, 1968). We have recently investigated an infant with abnormalities of both the sulfur amino acids and methylmalonic acid. This paper summarizes the results of our investigation which indicate the occurrence of a hitherto unrecognized metabolic abnormality, a defective ability to accumulate the coenzymatically active derivatives of B 12. As far as we are aware, this is the first proven instance of deranged vitamin metabolism in a human.

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