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An Alternate Metabolic Route for the Synthesis of Inosine 5′-Phosphate (IMP) in the Lesch-Nyhan Erythrocyte

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Purine Metabolism in Man—II

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB))

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Abstract

The cells of the Lesch-Nyhan individual, which are deficient in hypoxanthine-guanine phosphoribosyltransferase (HGPRT) activity, lack the ability to convert the 6-keto purines, hypoxanthine and guanine, to the corresponding nucleotides (1). Mammalian cells, in general, lack appreciable kinase activity for the 6-keto purine nucleosides, inosine and guanosine (2,3). Thus, the major route to IMP and GMP in the Lesch- Nyhan cell is via the overall pathway of de novo purine nucleotide synthesis in which the purine ring of IMP is built up on carbon 1 of a molecule of ribose 5-phosphate (4), The IMP is readily converted to GMP in normal human and Lesch-Nyhan cells.

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© 1977 Plenum Press, New York

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Lowy, B.A., Williams, M.K. (1977). An Alternate Metabolic Route for the Synthesis of Inosine 5′-Phosphate (IMP) in the Lesch-Nyhan Erythrocyte. In: Müller, M.M., Kaiser, E., Seegmiller, J.E. (eds) Purine Metabolism in Man—II. Advances in Experimental Medicine and Biology. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-4223-6_48

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  • DOI: https://doi.org/10.1007/978-1-4613-4223-6_48

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-4225-0

  • Online ISBN: 978-1-4613-4223-6

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