Document Detail

Adenosine deaminase messenger RNAs in lymphoblast cell lines derived from leukemic patients and patients with hereditary adenosine deaminase deficiency.
MedLine Citation:
PMID:  6134754     Owner:  NLM     Status:  MEDLINE    
Hereditary deficiency of adenosine deaminase (ADA) usually causes profound lymphopenia with severe combined immunodeficiency disease. Cells from patients with ADA deficiency contain less than normal, and sometimes undetectable, amounts of ADA catalytic activity and ADA protein. The molecular defects responsible for hereditary ADA deficiency are poorly understood. ADA messenger RNAs and their translation products have been characterized in seven human lymphoblast cell lines derived as follows: GM-130, GM-131, and GM-2184 from normal adults; GM-3043 from a partially ADA deficient, immunocompetent !Kung tribesman; GM-2606 from an ADA deficient, immunodeficient child; CCRF-CEM and HPB-ALL from leukemic children. ADA messenger (m)RNA was present in all lines and was polyadenylated. The ADA synthesized by in vitro translation of mRNA from each line reacted with antisera to normal human ADA and was of normal molecular size. There was no evidence that posttranslational processing of ADA occurred in normal, leukemic, or mutant lymphoblast lines. Relative levels of specific translatable mRNA paralleled levels of ADA protein in extracts of the three normal and two leukemic lines. However, unexpectedly high levels of ADA specific, translatable mRNA were found in the mutant GM-2606 and GM-3043 lines, amounting to three to four times those of the three normal lines. Differences in the amounts of ADA mRNA and rates of ADA synthesis appear to be of primary importance in maintaining the differences in ADA levels among lymphoblast lines with structurally normal ADA. ADA deficiency in at least two mutant cell lines is not caused by deficient levels of translatable mRNA, and unless there is some translational control of this mRNA, the characteristic cellular ADA deficiency is most likely secondary to synthesis and rapid degradation of a defective ADA protein.
G S Adrian; J J Hutton
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The Journal of clinical investigation     Volume:  71     ISSN:  0021-9738     ISO Abbreviation:  J. Clin. Invest.     Publication Date:  1983 Jun 
Date Detail:
Created Date:  1983-08-11     Completed Date:  1983-08-11     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  7802877     Medline TA:  J Clin Invest     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1649-60     Citation Subset:  AIM; IM    
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MeSH Terms
Adenosine Deaminase / biosynthesis,  deficiency*,  genetics
B-Lymphocytes / enzymology
Cell Line
Immunosorbent Techniques
Leukemia, Lymphoid / enzymology*
Nucleoside Deaminases / deficiency*
Poly A / metabolism
Protein Biosynthesis
RNA, Messenger / analysis,  metabolism*
T-Lymphocytes / enzymology
Grant Support
Reg. No./Substance:
0/RNA, Messenger; 24937-83-5/Poly A; EC 3.5.4.-/Nucleoside Deaminases; EC Deaminase

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