TY - JOUR
T1 - Metabolic abnormalities of human adenosine deaminase deficiency reproduced in the mouse by 2′-deoxycoformycin, an adenosine deaminase inhibitor
AU - Ratech, Howard
AU - Thorbecke, G. Jeanette
AU - Hirschhorn, Rochelle
N1 - Funding Information:
’ This work was supported by grants NIH AI 10343, and NIH AI 14829. Howard Ratech was supported by a scholarship from the Leopold Schepp Foundation and by NC1 Training Grant 5 T33 CA 09161. ” To whom reprint requests should be addressed.
PY - 1981/10
Y1 - 1981/10
N2 - Genetic absence of the enzyme adenosine deaminase in man results in severe combined immunodeficiency disease. Administration of 2′-deoxycoformycin, a tight-binding specific inhibitor of adenosine deaminase, to the mouse in vivo reproduces the metabolic abnormalities reported thus far in patients genetically lacking adenosine deaminase activity. These include markedly elevated excretion of 2′-deoxyadenosine, and inhibition of adenosine deaminase as well as of S-adenosyl homocysteine hydrolase tissue activities. Additionally there is a thymus-specific 10-fold increase in 2′-deoxyadenosine triphosphate content. This murine biochemical model of human adenosine deaminase deficiency should prove of value in studying the relationship of nucleoside metabolism to immunologic functional activity.
AB - Genetic absence of the enzyme adenosine deaminase in man results in severe combined immunodeficiency disease. Administration of 2′-deoxycoformycin, a tight-binding specific inhibitor of adenosine deaminase, to the mouse in vivo reproduces the metabolic abnormalities reported thus far in patients genetically lacking adenosine deaminase activity. These include markedly elevated excretion of 2′-deoxyadenosine, and inhibition of adenosine deaminase as well as of S-adenosyl homocysteine hydrolase tissue activities. Additionally there is a thymus-specific 10-fold increase in 2′-deoxyadenosine triphosphate content. This murine biochemical model of human adenosine deaminase deficiency should prove of value in studying the relationship of nucleoside metabolism to immunologic functional activity.
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U2 - 10.1016/0090-1229(81)90200-2
DO - 10.1016/0090-1229(81)90200-2
M3 - Article
C2 - 6268339
AN - SCOPUS:0019513431
SN - 0090-1229
VL - 21
SP - 119
EP - 127
JO - Clinical Immunology and Immunopathology
JF - Clinical Immunology and Immunopathology
IS - 1
ER -