GTP depletion and other erythrocyte abnormalities in inherited pnp deficiency

H. Anne Simmonds, Alan R. Watson, Dianne R. Webster, Amrik Sahota, David Perrett

Research output: Contribution to journalArticlepeer-review

24 Scopus citations

Abstract

GTP levels were low and NAD+ levels high in purine nucleoside phosphorylase (PNP) deficient erythrocytes, in addition to the raised deoxy-GTP (dGTP) levels previously noted by others. dGTP was also identified in the PNP deficient child's lymphocytes. A further novel finding was the conversion of hypoxanthine to inosine by the PNP deficient red cells, as compared to inosine monophosphate (IMP) in controls. This has been attributed to IMP formation with subsequent breakdown, and raises interesting questions regarding the controls which normally maintain erythrocyte nucleotide pools. These findings may also explain the gross purine overproduction seen in this defect; they may likewise be related to the associated immunodeficiency, anaemia, and other clinical manifestations. The results may also have important implications for the development and clinical use of PNP inhibitors.

Original languageEnglish (US)
Pages (from-to)941-946
Number of pages6
JournalBiochemical Pharmacology
Volume31
Issue number6
DOIs
StatePublished - Mar 15 1982
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Pharmacology

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