Biochemical bases of accelerated purine biosynthesis de novo in human fibroblasts lacking hypoxanthine-guanine phosphoribosyltransferase.
作者： F M Rosenbloom , J F Henderson , I C Caldwell , W N Kelley , J E Seegmiller
DOI: 10.1016/S0021-9258(19)56968-X
关键词:
摘要: Fibroblasts cultured from patients completely deficient in hypoxanthine-guanine phosphoribosyltransferase activity were studied to determine the biochemical bases of increased rate purine biosynthesis de novo observed such patients. Enzyme-deficient fibroblasts had accelerated rates this pathway and elevated levels 5-phosphoribosyl 1-pyrophosphate, although synthesis compound was not increased. These cells sensitive feedback inhibition by nucleotides adenine 6-methylmercaptopurine ribonucleoside, but hypoxanthine guanine. The concentrations ribonucleotides mutant normal. catabolism inosinate synthesized enzyme-deficient fibroblasts.
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