期刊
EXPERIMENTAL HEMATOLOGY
卷 30, 期 5, 页码 410-420出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/S0301-472X(02)00782-8
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资金
- NHLBI NIH HHS [R37 HL32987, P01 HL53586, K08 HL04071] Funding Source: Medline
- NIDDK NIH HHS [P50 DK49218] Funding Source: Medline
Objective. The aim of this study was to develop a rapid laboratory procedure that is capable of subtyping Fanconi anemia (FA) complementation groups FA-A, FA-C, FA-G, and FA-non-ACG patients from a small amount of peripheral blood. Materials and Methods. For this test, primary peripheral blood-derived FAT cells were transduced with oncoretroviral vectors that expressed FANCA, FANCC, or FANCG cDNA. We achieved a high efficiency of gene transfer into primary FA T cells by using the fibronectin fragment CH296 during transduction. Transduced cells were analyzed for correction of the characteristic DNA cross-linker hypersensitivity by, cell survival or by metaphase analyses. Results. Retroviral vectors containing the cDNA for FA-A, FA-C, and FA-G, the most frequent complementation groups in North America, allowed rapid identification of the defective gene by complementation of primary T cells from 12 FA patients. Conclusion. Phenotypic correction of FA T cells using retroviral vectors can be used successfully to determine the FA complementation group immediately after diagnosis of the disease. (C) 2002 International Society for Experimental Hematology. Published by Elsevier Science Inc.
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