Severe pyridine nucleotide depletion in fibroblasts from Lesch–Nyhan patients
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منابع مشابه
Severe pyridine nucleotide depletion in fibroblasts from Lesch-Nyhan patients.
The relationship between a complete deficiency of the purine enzyme hypoxanthine-guanine phosphoribosyltransferase and the neurobehavioural abnormalities in Lesch-Nyhan disease remains an enigma. In vitro studies using lymphoblasts or fibroblasts have evaluated purine and pyrimidine metabolism with conflicting results. This study focused on pyridine nucleotide metabolism in control and Lesch-Ny...
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The isolation and purification of a soluble transhydrogenase from Chromatium is described. The enzyme promotes the oxidation of reduced triphosphopyridine nucleotide by diphosphopyridine nucleotide. However, it will also use several analogues of DPN and TPN which include deamino-DPN, deamino-TPN, thionicotinamide DPN, and deamino-DPNH. Nicotinamide mononucleotide was also reduced. The enzyme do...
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established unequivocally that either a hydrogen or electron transfer was involved in this reaction. The possibility of a transfer of the monoester phosphate group or an exchange of reduced and oxidized nicotinamidecontaining moieties (nucleoside, nucleotide, etc.) was ruled out by this and other experiments. There still remained, however, the problem of distinguishing experimentally between th...
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The synthesis of NAD and NADP by rat adipose tissue was measured in vitro. Nicotinamide-7-14C and NaHPP04 were incorporated together into NAD with a =P/14C ratio of 1.82 and nicotinic-7-14C acid and NaH232POa with a ratio of 1.94. Nicotinic acid stimulated, by 90%, lipogenesis from glucose-UW by rat adipose tissue in vitro. Glucose plus insulin and refeeding for 48 hr after a 48 hr fast markedl...
متن کاملA reduced pyridine nucleotide pyrophosphatase.
Reaction 1 is catalyzed by DPNase and has been observed in higher animals (1, 2), in Neurospora (3), and in some bacteria (4). Reaction 2 is catalyzed by a deaminase found in Taka-Diastase (5). Reaction 3 is the DPN pyrophosphorylase from hog liver, as described by Kornberg (6) ; it is the only reversible reaction of those shown. The pyrophosphatase splitting shown in Reaction 4 has been observ...
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ژورنال
عنوان ژورنال: Biochemical Journal
سال: 2002
ISSN: 0264-6021,1470-8728
DOI: 10.1042/bj20020148