In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients.
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Adenosine Deaminase Deficiency - More Than Just an ImmunodeficiencyAn enzyme captured in two conformational states: crystal structure of S-adenosyl-L-homocysteine hydrolase from Bradyrhizobium elkaniiAdenosine deaminase deficiency increases thymic apoptosis and causes defective T cell receptor signalingCovalent labelling of ligand binding sites of human placental S-adenosylhomocysteine hydrolase with 8-azido derivatives of adenosine and cyclic AMPDecreased methionine synthesis in purine nucleoside-treated T and B lymphoblasts and reversal by homocysteineBone marrow transplantation only partially restores purine metabolites to normal in adenosine deaminase-deficient patientsMetabolic consequences of adenosine deaminase deficiency in mice are associated with defects in alveogenesis, pulmonary inflammation, and airway obstructionAdenosine deaminase: functional implications and different classes of inhibitors.Autoimmune dysregulation and purine metabolism in adenosine deaminase deficiency.Human purine metabolism: some recent advances and relationships with immunodeficiencyNovel splicing, missense, and deletion mutations in seven adenosine deaminase-deficient patients with late/delayed onset of combined immunodeficiency disease. Contribution of genotype to phenotype.S-adenosylhomocysteine hydrolase deficiency in a human: a genetic disorder of methionine metabolismEcto-5'-nucleotidase activity in human T cell subsets. Decreased numbers of ecto-5'-nucleotidase positive cells from both OKT4+ and OKT8+ cells in patients with hypogammaglobulinemia.Central nervous system dysfunction and erythrocyte guanosine triphosphate depletion in purine nucleoside phosphorylase deficiencyConversion of a stem cell leukemia from a T-lymphoid to a myeloid phenotype induced by the adenosine deaminase inhibitor 2'-deoxycoformycin.Resistance of an adenosine kinase-deficient human lymphoblastoid cell line to effects of deoxyadenosine on growth, S-adenosylhomocysteine hydrolase inactivation, and dATP accumulation.Modulation of cyclic AMP metabolism by S-adenosylhomocysteine and S-3-deazaadenosylhomocysteine in mouse lymphocytes.ATP depletion as a consequence of adenosine deaminase inhibition in man.Altered intracellular and extracellular signaling leads to impaired T-cell functions in ADA-SCID patients.S-adenosylhomocysteine hydrolase deficiency in a 26-year-old man.S-Adenosylhomocysteine hydrolase deficiency: a second patient, the younger brother of the index patient, and outcomes during therapyProposed explanation for S-adenosylhomocysteine hydrolase deficiency in purine nucleoside phosphorylase and hypoxanthine-guanine phosphoribosyltransferase-deficient patientsBiochemical and functional abnormalities in lymphocytes from an adenosine deaminase-deficient patient during enzyme replacement therapy.Inactivation of S-adenosylhomocysteine hydrolase during adenine arabinoside therapyRestoration of adenosine deaminase-deficient human thymocyte development in vitro by inhibition of deoxynucleoside kinasesInsights into thymic purine metabolism and adenosine deaminase deficiency revealed by transgenic mice overexpressing ecto-5'-nucleotidase (CD73).Overview of possible relation of defects in purine metabolism to immune deficiency.Erythrocyte disorders of purine and pyrimidine metabolism.Inactivation of liver S-adenosylhomocysteine hydrolase in vitro of rats treated with erythro-9-(2-hydroxynon-3-yl)adenine.S-Adenosylhomocysteine hydrolase from human placenta. Affinity purification and characterization.Adenosine Deaminase (ADA)-Deficient Severe Combined Immune Deficiency (SCID): Molecular Pathogenesis and Clinical Manifestations.
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P2860
In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on April 1979
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
In vivo inactivation of erythr ...... deaminase-deficient patients.
@en
In vivo inactivation of erythr ...... deaminase-deficient patients.
@nl
type
label
In vivo inactivation of erythr ...... deaminase-deficient patients.
@en
In vivo inactivation of erythr ...... deaminase-deficient patients.
@nl
prefLabel
In vivo inactivation of erythr ...... deaminase-deficient patients.
@en
In vivo inactivation of erythr ...... deaminase-deficient patients.
@nl
P2093
P2860
P356
P1476
In vivo inactivation of erythr ...... deaminase-deficient patients.
@en
P2093
P2860
P304
P356
10.1172/JCI109367
P407
P577
1979-04-01T00:00:00Z