Title: Molybdopterin Biosynthesis in Man. Properties of the Converting Factor in Liver Tissue from a Molybdenum Cofactor Deficient Patient
Abstract: Molybdenum cofactor deficiency is an inborn error of metabolism resulting from an inability to synthesize functional molybdopterin, the organic pterin component of the molybdenum cofactor.1 Cofactor deficient patients exhibit combined deficiencies of the three known molyboenzymes in man, sulfite oxidase, xanthine dehydrogenase and aldehyde oxidase. Molybdopterin biosynthesis requires a multistep pathway, and patients with defects at two different points in the pathway comprise two identified complementation groups.2 The terminal step in molybdopterin biosynthesis is carried out by a protein, termed converting factor, that uses as its substrate a desulfo, cyclic phosphopterin, precursor Z.3 The reaction catalyzed by the converting factor and the structures of precursor Z, molybdopterin and the molybdenum cofactor are outlined in Figure 1.
Publication Year: 1993
Publication Date: 1993-01-01
Language: en
Type: article
Indexed In: ['crossref', 'pubmed']
Access and Citation
Cited By Count: 2
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