The expression of hHR21sp gene in the peripheral blood cells and bone marrow cells of patients with Fanconi anemia

AIM:To detect the expression of hHR21^spgene in normal human peripheral blood cells and in hemapoietic cell from fanconi anemia(FA) patients bone marrow after exposure to UV and gamma-irradiation.METHODS:RNA of FA hemapoietic cell and normal peripheral blood cells were harvested after UV and gamma irradiation. hHR21^sp gene expression was analyzed using RT-PCR, southern blot hybridization, phosphoimmage autoradiogram.RESULTS:The expression of hHR21^sp gene in PBL varied with the time of UV gamma-irradiation and increased obviously six hours after 80 J/M² UV-or 5 Gy gamma-irradiation. However, the expression was down-regulated in FA hemapoietic cell from FA patients indicating that hHR21^sp expression is something related to the ability of cells to repair DNA double strand break.CONCLUSION:The expression of hHR21^sp down-regulated in the FA syndrome and this might be involved in the pathogenesis of the FA.