Purine nucleoside phosphorylase deficiency.

ML Markert - Immunodeficiency reviews, 1991 - europepmc.org
Purine nucleoside phosphorylase (PNP) deficiency is a rare inherited disease accounting
for approximately 4% of patients with severe combined immunodeficiency. Thirty-three …

Metabolic basis for disorders of purine nucleotide degradation

IH Fox - Metabolism, 1981 - Elsevier
Purine nucleotide degradation refers to a regulated series of reactions by which human
purine ribonucleotides and deoxyribonucleotides are degraded to uric acid in humans. Two …

Crystal structure of the ternary complex of E. coli purine nucleoside phosphorylase with formycin B, a structural analogue of the substrate inosine, and phosphate …

G Koellner, M Luić, D Shugar, W Saenger… - Journal of molecular …, 1998 - Elsevier
The ternary complex of purine nucleoside phosphorylase from E. coli with formycin B and a
sulphate or phosphate ion crystallized in the hexagonal space group P6122 with unit cell …

Purine metabolism in relation to leukemia and lymphoid cell differentiation

J Van Laarhoven, CHMM De Bruyn - Leukemia research, 1983 - Elsevier
A number of inborn errors of purine metabolism have been associated with
immunodeficiency diseases. From studies to the possible mechanism (s) leading to the …

Calf spleen purine nucleoside phosphorylase: purification, sequence and crystal structure of its complex with an N (7)-acycloguanosine inhibitor

A Bzowska, M Luić, W Schröder, D Shugar, W Saenger… - FEBS letters, 1995 - Elsevier
Calf spleen purine nucleoside phosphorylase was purified to homogeneity and its amino
acid sequence was determined. The complex of the enzyme with an N (7)-acycloguanosine …

Purine nucleoside phosphorylase

JD Stoeckler, RE Parks Jr - Methods Used in Adenosine …, 2013 - books.google.com
It has long been appreciated that purine nucleoside phosphorylase (PNP; purine
nucleoside: orthophosphate ribosyltransferase, EC 2.4. 2.1) may play a role in cancer …

Inhibitors of purine nucleoside phosphorylase: effects of 9-deazapurine ribonucleosides and synthesis of 5′-deoxy-5′-iodo-9-deazainosine

JD Stoeckler, JB Ryden, RE Parks Jr, MY Chu, MI Lim… - Cancer research, 1986 - AACR
Deazapurine ribonucleosides constitute a new class of noncleavable purine nucleoside
phosphorylase inhibitors that have at least 30-fold greater affinity for the enzyme than the …

Giardiasis and immunodeficiency diseases.

ADB Webster - 1981 - cabidigitallibrary.org
The possibility that patients with primary immunodeficiency diseases, such as primary
hypogammaglobulinaemia, are especially susceptible to giardiasis is discussed. Although …

A new case of purine nucleoside phosphorylase deficiency: enzymologic, clinical, and immunologic characteristics

G Rijksen, W Kuis, SK Wadman, LJM Spaapen… - Pediatric …, 1987 - nature.com
Deficiency of purine nucleoside phosphorylase (PNP) was detected in a 3-yr-oId boy who
was admitted for investigation of a behavior disorder and spastic diplegia. The urinary …

Adenosine deaminase and purine nucleoside phosphorylase deficiencies: evaluation of therapeutic interventions in eight patients

ML Markert, MS Hershfield, RI Schiff… - Journal of clinical …, 1987 - Springer
The courses of six patients with adenosine deaminase (ADA) and two with purine
nucleoside phosphorylase (PNP) deficiencies were evaluated before and after therapy. The …