Purine nucleoside phosphorylase deficiency associated with selective cellular immunodeficiency.
about
Point mutations at the purine nucleoside phosphorylase locus impair thymocyte differentiation in the mouseImmucillin H, a powerful transition-state analog inhibitor of purine nucleoside phosphorylase, selectively inhibits human T lymphocytesMolecular analysis of mutations in a patient with purine nucleoside phosphorylase deficiencyDeoxyadenosine triphosphate as a mediator of deoxyguanosine toxicity in cultured T lymphoblastsThe metabolism of deoxyguanosine and guanosine in human B and T lymphoblasts. A role for deoxyguanosine kinase activity in the selective T-cell defect associated with purine nucleoside phosphorylase deficiencyStructural analyses reveal two distinct families of nucleoside phosphorylasesB cells as well as T cells form deoxynucleotides from either deoxyadenosine or deoxyguanosineErythrocyte metabolism in purine nucleoside phosphorylase deficiency after enzyme replacement therapy by infusion of erythrocytesMitochondrial basis for immune deficiency. Evidence from purine nucleoside phosphorylase-deficient miceGene and protein expression profiling of the microvascular compartment in experimental autoimmune encephalomyelitis in C57Bl/6 and SJL mice.Combined familial adenosine deaminase and purine nucleoside phosphorylase deficiencies.A proof-of-principle pharmacokinetic, pharmacodynamic, and clinical study with purine nucleoside phosphorylase inhibitor immucillin-H (BCX-1777, forodesine)Purinogenic immunodeficiency diseases: selective toxicity of deoxyribonucleosides for T cells.Adenosine deaminase and purine nucleoside phosphorylase activities in peripheral lymphocytes from patients with solid tumours.Metabolism of purines in macrophages. Effect of functional state of the cells.Inborn errors of molybdenum metabolism: combined deficiencies of sulfite oxidase and xanthine dehydrogenase in a patient lacking the molybdenum cofactor.Purinogenic immunodeficiency diseases. Differential effects of deoxyadenosine and deoxyguanosine on DNA synthesis in human T lymphoblasts.Purine nucleoside phosphorylase deficiency. Evidence for molecular heterogeneity in two families with enzyme-deficient members.Deoxyguanosine triphosphate as a possible toxic metabolite in the immunodeficiency associated with purine nucleoside phosphorylase deficiency.Selective toxicity of purine deoxynucleosides for human lymphocyte growth and function.Adenosine kinase initiates the major route of ribavirin activation in a cultured human cell line.Purine metabolism in cultured human fibroblasts derived from patients deficient in hypoxanthine phosphoribosyltransferase, purine nucleoside phosphorylase, or adenosine deaminase.Inborn errors of metabolism underlying primary immunodeficiencies.Purine nucleoside phosphorylase deficiency associated with a fatal lymphoproliferative disorder.Immune deficiency due to adenosine deaminase and purine nucleoside phosphorylase deficiency: a simple diagnostic test.Partial purine nucleoside phosphorylase deficiency. Studies of lymphocyte function.Infusion of Sibling Marrow in a Patient with Purine Nucleoside Phosphorylase Deficiency Leads to Split Mixed Donor Chimerism and Normal Immunity.Mechanisms of 2'-deoxyguanosine toxicity in mouse T-lymphoma cells with purine nucleoside phosphorylase deficiency and resistance to inhibition of ribonucleotide reductase by dGTP.Lack of inhibition of purine nucleoside phosphorylase and adenosine deaminase in patients treated with azathioprine.Transition state analogue inhibitors of purine nucleoside phosphorylase from Plasmodium falciparum.Familial spastic paraplegia as the presenting manifestation in patients with purine nucleoside phosphorylase deficiency.Neurotoxic side effects in children with refractory or relapsed T-cell malignancies treated with nelarabine based therapy.Lymphocyte purine metabolism -- significance of 5'-nucleotidase deficiency: a review
P2860
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P2860
Purine nucleoside phosphorylase deficiency associated with selective cellular immunodeficiency.
description
1977 nî lūn-bûn
@nan
1977年の論文
@ja
1977年論文
@yue
1977年論文
@zh-hant
1977年論文
@zh-hk
1977年論文
@zh-mo
1977年論文
@zh-tw
1977年论文
@wuu
1977年论文
@zh
1977年论文
@zh-cn
name
Purine nucleoside phosphorylas ...... ive cellular immunodeficiency.
@en
type
label
Purine nucleoside phosphorylas ...... ive cellular immunodeficiency.
@en
prefLabel
Purine nucleoside phosphorylas ...... ive cellular immunodeficiency.
@en
P2093
P1476
Purine nucleoside phosphorylas ...... ive cellular immunodeficiency.
@en
P2093
Ballieux RE
Hendrickx GF
de Bree PK
van Heukelom LH
P304
P356
10.1056/NEJM197703242961203
P407
P577
1977-03-01T00:00:00Z