scholarly journals Severe Combined Immunodeficiency Disease with Adenosine Deaminase Deficiency

1979 ◽  
Vol 129 (2) ◽  
pp. 197-202 ◽  
Author(s):  
SHINKICHI YOKOYAMA ◽  
TADASHI HAYASHI ◽  
YOZO YOSHIMURA ◽  
KIYOSHI IRIMADA ◽  
TETSU SAITO ◽  
...  
Keyword(s):  
Adenosine Deaminase ◽  
Severe Combined Immunodeficiency ◽  
Combined Immunodeficiency ◽  
Adenosine Deaminase Deficiency ◽  
Immunodeficiency Disease ◽  
Severe Combined Immunodeficiency Disease

scholarly journals Studies on the effect of deoxyadenosine on deoxycoformycin-treated myeloid and lymphoid stem cells

Blood ◽  
1982 ◽  
Vol 60 (4) ◽  
pp. 872-876
Author(s):  
MT Aye ◽  
JV Dunne ◽  
WC Yang
Keyword(s):  
Stem Cells ◽  
Adenosine Deaminase ◽  
Severe Combined Immunodeficiency ◽  
Immune Dysfunction ◽  
Model Systems ◽  
Hemopoietic Stem Cells ◽  
Combined Immunodeficiency ◽  
Proliferation And Differentiation ◽  
Immunodeficiency Disease ◽  
Severe Combined Immunodeficiency Disease

Adenosine deaminase (ADA) deficiency has been reported in association with severe combined immunodeficiency disease (SCID). The mechanism by which ADA deficiency causes immune dysfunction has been investigated in model systems to which the ADA inhibitor deoxycoformycin (dCf) had been added. Previously, we demonstrated that dCF did not prevent proliferation and differentiation of myeloid and lymphoid stem cells. We have now shown that addition of deoxyadenosine to dCf-containing cultures inhibited proliferation of hemopoietic stem cells. This inhibition was, however, equally effective for both normal myeloid and lymphoid stem cells. These findings suggest that other differences may exist between SCID myeloid and lymphoid stem cells to account for the relative sparing of myelopoiesis in SCID patients.

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