Phenotypic correction of factor IX deficiency in skin fibroblasts of hemophilic dogs.
Phenotypic correction of factor IX deficiency in skin fibroblasts of hemophilic dogs.
复制标题
血友病犬皮肤成纤维细胞中因子 IX 缺乏的表型校正。
DOI：
10.1073/pnas.87.13.5173
复制
发表时间：
1990
影响因子：
11.1
通讯作者：
Verma,IM
中科院分区：
文献类型：
--
作者：
Axelrod,JH;Read,MS;Brinkhous,KM;Verma,IM
用含有犬因子IX cDNA的重组逆转录病毒（LNCdF 9 L）转导血友病犬的原代皮肤成纤维细胞。培养基中分泌高水平的生物活性犬因子IX（1.0 μ g/10（6）个细胞/24 h）。如果在感染期间用碱性成纤维细胞生长因子刺激细胞，则产生的因子IX的水平显著增加。此外，我们还报告说，这种病毒转导的内皮细胞可以产生高水平的生物活性因子IX。我们建议，皮肤成纤维细胞和内皮细胞从血友病B狗可能作为潜在的场地开发和测试模型治疗血友病B的逆转录病毒介导的基因替代疗法。
Primary skin fibroblasts from hemophilic dogs were transduced by recombinant retrovirus (LNCdF9L) containing a canine factor IX cDNA. High levels of biologically active canine factor IX (1.0 micrograms per 10(6) cells per 24 hr) were secreted in the medium. The level of factor IX produced increased substantially if the cells were stimulated by basic fibroblast growth factor during infection. Additionally, we also report that endothelial cells transduced by this virus can produce high levels of biologically active factor IX. We propose that skin fibroblasts and endothelial cells from hemophilia B dogs may serve as potential venues for the development and testing of models for treatment of hemophilia B by retrovirally mediated gene replacement therapy.