Comparative characterization of B-lymphoblastoid cell lines in adenosine deaminase deficiency and its heterozygote.
B-lymphoblastoid cell lines (LCL) transformed by Epstein-Barr virus (EBV) were established from a patient with severe combined immunodeficiency (SCID) caused by adenosine deaminase (ADA) deficiency, from his mother and from a normal volunteer. ADA activities of the patient's and mother's LCL were about 0.5% and 25% of that of the normal LCL, respectively. Proliferation of these three LCL was related to the ADA activity. The patient's LCL grew well in medium containing fetal bovine serum (FBS), but slowly in medium with the ADA inhibitor 2'-deoxycoformycin-treated FBS, horse serum, or without serum. Proliferation of the patient's LCL was markedly inhibited in serum-free medium containing 2'-deoxyadenosine.
Sawami
H
H
Toyokawa
Y
Y
Ito
K
K
Uchino
H
H
D000243:Adenosine Deaminase / Q000172:deficiency*; D001402:B-Lymphocytes / Q000166:cytology*; D002455:Cell Division; D002461:Cell Line, Transformed; D005260:Female; D006579:Heterozygote; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000201:enzymology* / Q000235:genetics / Q000473:pathology; D008297:Male; D009700:Nucleoside Deaminases / Q000172:deficiency*
D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase