Metabolic and Immunologic Consequences of Limited Adenosine Deaminase Expression in Mice

ADENOSINE-DEAMINASE DEFICIENCY IN TWO PATIENTS WITH SEVERELY IMPAIRED CELLULAR IMMUNITY

1972 • 1,654 citations

T-cell apoptosis detected in situ during positive and negative selection in the thymus

1994 • 1,071 citations

Lymphospecific toxicity in adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency: Possible role of nucleoside kinase(s)

1977 • 416 citations

Deoxyadenosine triphosphate as a potentially toxic metabolite in adenosine deaminase deficiency.

1978 • 399 citations

Apparent suicide inactivation of human lymphoblast S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine and adenine arabinoside. A basis for direct toxic effects of analogs of adenosine.

1979 • 277 citations

Identification and quantitation of adenine deoxynucleotides in erythrocytes of a patient with adenosine deaminase deficiency and severe combined immunodeficiency.

1978 • 277 citations

Deoxyadenosine metabolism and cytotoxicity in cultured mouse T lymphoma cells: a model for immunodeficiency disease

1978 • 230 citations

Adenosine receptors.

1992 • 196 citations

In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients.

1979 • 168 citations

Disruption of the adenosine deaminase gene causes hepatocellular impairment and perinatal lethality in mice.

1995 • 146 citations

Adenosine receptor-mediated accumulation of cyclic AMP-induced T-lymphocyte death through internucleosomal DNA cleavage.

1990 • 137 citations

Evidence for a complex regulatory array in the first intron of the human adenosine deaminase gene.

1989 • 124 citations

Adenosine–deaminase–deficient mice die perinatally and exhibit liver–cell degeneration, atelectasis and small intestinal cell death

1995 • 117 citations

Overview of Biochemical Abnormalities and Molecular Genetics of Adenosine Deaminase Deficiency

1993 • 99 citations

Novel splicing, missense, and deletion mutations in seven adenosine deaminase-deficient patients with late/delayed onset of combined immunodeficiency disease. Contribution of genotype to phenotype.

1993 • 98 citations

An immunomorphologic study of adenosine deaminase distribution in human thymus tissue, normal lymphocytes, and hematopoietic cell lines.

1981 • 87 citations

Coordinate developmental regulation of purine catabolic enzyme expression in gastrointestinal and postimplantation reproductive tracts.

1991 • 84 citations

Effects of (R)‐deoxycoformycin (pentostatin) on intrauterine nucleoside catabolism and embryo viability in the pregnant mouse

1992 • 78 citations

p53 expression is required for thymocyte apoptosis induced by adenosine deaminase deficiency.

1995 • 76 citations

Differences in deoxyadenosine metabolism in human and mouse lymphocytes.

1980 • 71 citations

Inhibition of Methylation by Adenosine in Adenosine Deaminase-Inhibited, Phytohemagglutinin-Stimulated Human Lymphocytes

1979 • 69 citations

Heterogeneity of biochemical, clinical and immunological parameters in severe combined immunodeficiency due to adenosine deaminase deficiency.

1987 • 65 citations

A biochemical genetic study of the role of specific nucleoside kinases in deoxyadenosine phosphorylation by cultured human cells.

1981 • 59 citations

Tissue-specific Rescue Suggests That Placental Adenosine Deaminase Is Important for Fetal Development in Mice

1995 • 55 citations

Activation of apoptosis in early mouse embryos by 2′‐deoxyadenosine exposure

1994 • 53 citations

Ontogeny of Adenosine Deaminase in the Mouse Decidua and Placenta: Immunolocalization and Embryo Transfer Studies1

1991 • 49 citations

5' flanking sequences of the murine adenosine deaminase gene direct expression of a reporter gene to specific prenatal and postnatal tissues in transgenic mice.

1992 • 42 citations

Animal model for immune dysfunction associated with adenosine deaminase deficiency.

1980 • 36 citations

Inactivation of S-adenosyl-L-homocysteine hydrolase by cAMP results from dissociation of enzyme-bound NAD+.

1985 • 29 citations

Decreased S-adenosylhomocysteine hydrolase in inborn errors of purine metabolism.

1980 • 28 citations

Proposed explanation for S-adenosylhomocysteine hydrolase deficiency in purine nucleoside phosphorylase and hypoxanthine-guanine phosphoribosyltransferase-deficient patients.

1981 • 23 citations

Metabolic abnormalities of human adenosine deaminase deficiency reproduced in the mouse by 2′-deoxycoformycin, an adenosine deaminase inhibitor

1981 • 19 citations

Effects of deoxycoformycin in mice. III. A murine model reproducing multi-system pathology of human adenosine deaminase deficiency.

1985 • 16 citations

Adenosine Deaminase and Thymocyte Maturation

1991 • 13 citations