Citations to this article

Dietary-Induced Variation of Hypoxanthine-Guanine Phosphoribosyl Transferase Activity in Patients with the Lesch-Nyhan Syndrome
William J. Arnold, William N. Kelley
William J. Arnold, William N. Kelley
Published April 1, 1973
Citation Information: J Clin Invest. 1973;52(4):970-973. https://doi.org/10.1172/JCI107263.
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Dietary-Induced Variation of Hypoxanthine-Guanine Phosphoribosyl Transferase Activity in Patients with the Lesch-Nyhan Syndrome

Abstract

We have studied three patients with the Lesch-Nyhan syndrome to assess the effect of dietary purines on erythrocyte hypoxanthine-guanine phosphoribosyltransferase (HGPRT) activity. During dietary purine restriction HGPRT activity rose in all three patients; resumption of normal dietary purine intake or the addition of adenine (10 mg/kg per day) to a purinefree diet resulted in a fall in HGPRT activity. These changes in enzyme activity appeared to be due to an activation or inactivation of the mutant enzyme without a change in the half-life or absolute amount of HGPRT enzyme protein.

Authors

William J. Arnold, William N. Kelley

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Year: 2022 2006 1986 1983 1978 1976 1974 Total
Citations: 1 1 1 1 1 2 1 8
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Citations to this article (8)

Title and authors Publication Year
Sphinganine is associated with 24-h MAP in the non-sleepy with OSA
V Pak, K Russell, Z Shi, Q Zhang, J Cox, K Uppal, T Yu, V Hertzberg, K Liu, O Ioachimescu, N Collop, D Bliwise, N Kutner, A Rogers, S Dunbar 		Metabolomics : Official journal of the Metabolomic Society 2022
Advances in Enzymology - and Related Areas of Molecular Biology
H Waelsch 		Advances in Enzymology and Related Areas of Molecular Biology 2006
Genetic Disorders and the Fetus
A Milunsky 		1986
Elevated erythrocyte phosphoribosylpyrophosphate and ATP concentrations in Japanese sumo wrestlers
Y Nishida, I Akaoka, E Hayashi, T Miyamoto 		British Journal of Nutrition 1983
Uric Acid
WN Kelley, IM Weiner 		1978
Inborn Errors of Purine and Pyrimidine Metabolism
IH Fox 		Clinics in Perinatology 1976
Advances in Human Genetics 6
H Harris, K Hirschhorn 		1976
Purine Metabolism in Man
O Sperling, AD Vries, JB Wyngaarden 		1974

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