12 PRPP AND PURINE NUCLEOTIDE METABOLISM IN HUMAN LYMPHOBLASTS WITH BOTH PRPP SYNTHETASE SUPERACTIVITY AND HGPRT DEFICIENCY
نویسندگان
چکیده
منابع مشابه
Selective expression of phosphoribosylpyrophosphate synthetase superactivity in human lymphoblast lines.
Phenotypic expression of 5-phosphoribosyl 1-pyrophosphate (PRPP) synthetase superactivity was examined in lymphoblast lines derived from six unrelated male patients. Fibroblasts from these individuals have increased rates of PRPP and purine nucleotide synthesis and express four classes of kinetic derangement underlying enzyme superactivity: increased maximal reaction velocity (catalytic defect)...
متن کاملDe novo synthesis of purine nucleotides in human peripheral blood leukocytes. Excessive activity of the pathway in hypoxanthine-guanine phosphoribosyltransferase deficiency.
Human peripheral blood leukocytes were studied for the presence and the regulatory properties of the pathway of de novo synthesis of purine nucleotides. The cells were found to incorporate the labeled precursors formate and glycine into purines. The rate of [14C]-formate incorporation was decreased by several compounds known to inhibit purine synthesis by affecting the activity by glutamine pho...
متن کاملIn vivo effect of mutations at the PRPP binding site of the Bacillus subtilis purine repressor.
The Bacillus subtilis PurR mediates adenine repression and guanosine induction of purA. PRPP inhibits binding of PurR to DNA in vitro. Mutations in the PRPP binding motif of PurR caused strong repression regardless of purine exclusions or additions, establishing the role of PRPP as regulator of PurR.
متن کاملFeedback-Resistant Phosphoribosylpyrophosphate Synthetase Associated with Purine Overproduction and Gout PHOSPHORIBOSYLPYROPHOSPHATE AND PURINE METABOLISM
other family members investigated. The fibroblast studA preliminary report was presented at the Annual Meeting of the Israeli Association of Chemical Pathology, Rehovot, Israel, March 1974. Professor de Vries is an Established Investigator of the Chief Scientist's Bureau, Ministry of Health. Received for publication 6 February 1975 and in revised form 7 July 1975. ies demonstrate the following ...
متن کاملFeedback-Resistant Phosphoribosylpyrophosphate Synthetase Associated with Purine Overproduction and Gout PHOSPHORIBOSYLPYROPHOSPHATE AND PURINE METABOLISM
other family members investigated. The fibroblast studA preliminary report was presented at the Annual Meeting of the Israeli Association of Chemical Pathology, Rehovot, Israel, March 1974. Professor de Vries is an Established Investigator of the Chief Scientist's Bureau, Ministry of Health. Received for publication 6 February 1975 and in revised form 7 July 1975. ies demonstrate the following ...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1988
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-198807000-00036