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Purine nucleoside phosphorylase (PNP)

Sakiyama, T.

Nihon Rinsho. Japanese Journal of Clinical Medicine 54(12): 3220-3225

1996

ISSN/ISBN: 0047-1852
PMID: 8976095
Document Number: 456822
PNP, encoded by 6 exons on human chromosome 14q13, is a homotrimetic enzyme of approximately 96 k dalton. This enzyme reversibly catalyzes the phosphorolysis of purine nucleoside to their respective purine bases and the corresponding pentose-1-phosphate. The ultimate degradative product of purine nucleoside is uric acid, which is a depleted product with the absence of PNP activity. This leads to accumulation of deoxy-guanosine triphosphate, which inhibits the enzyme ribonucleoside reductase resulting in DNA synthesis block and T cell proliferation. Basic and brief knowledge of PNP biochemistry and functional physiology is summarized for the understanding of clinical features of PNP deficiency.

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