BLM Antibody
货号：
2742S 基本售价：
3360.0 元 规格：
-
产品信息
概述| 货号 | 2742S |
| 反应种属 | Human |
| 来源宿主 | Rabbit |
| 应用 | W |
| 使用方法 | WB(1:1000) |
性能| 供应商 | CST |
| 背景 | BLM, a member of the RecQ family of DNA helicases, is part of the BRCA1-associated genome surveillance complex (BASC) that responds to DNA damage, stalled replication forks and S phase arrest (1-4). Phosphorylation of BLM helicase at Thr99 and Thr122 occurs in response to genotoxic stress (4), and phosphorylation of Ser144 appears to be important in regulating chromosome stability during mitosis (5). Typical BLM protein resides in the nucleus and forms part of a dynamic protein complex that acts in response to DNA damage during specific periods of the cell cycle (6). Although RecQ helicases are rarely considered as essential enzymes, they function at the interface between DNA recombination and repair and are required for global genome stability maintenance. Mutations in BLM helicase are responsible for development of Bloom Syndrome, a recessive genetic disorder clinically characterized by short stature, immunodeficiency and elevated risk of malignancy (7). Similar alterations to genes encoding the related RecQ helicases RecQ4 and WRN also result in recessive genetic disorders associated with genomic instability (8,9). Cells from Bloom Syndrome patients exhibit genomic instability and increased frequency of sister chromatid exchange (10).BLM作为DNA解旋酶RecQ家族的一员，是BRCA1相关基因组监视复合体(BASC)的一部分，该复合体响应DNA损伤、复制叉停滞和S期阻滞(1-4)。BLM解旋酶99位和122位的苏氨酸磷酸化响应基因毒性应激反应(4)，而144位丝氨酸的磷酸化在细胞有丝分裂过程中调节染色体的稳定性(5)。典型的BLM蛋白位于细胞核内，组成了动力蛋白复合体的一部分，在细胞周期的特定时期响应DNA损伤(6)。尽管很少有人认为RecQ解旋酶是必不可少的酶类，但是它们在DNA重组和修复的界点发挥作用，并影响着总体基因组稳定性的维持。BLM解旋酶的突变是Bloom综合征的重要原因，该病属于隐性遗传病，临床特征有身材矮小、免疫缺陷和恶性肿瘤风险升高(7)。相类似的RecQ解旋酶RecQ4和WRN的基因编码的改变也导致与基因组不稳定性相关的隐性遗传性疾病(8,9)。取自Bloom综合征患者的细胞表现出基因组不稳定性和姐妹染色单体交换的频率增加(10)。 |
| 存放说明 | -20C |
| 计算分子量 | 190 |
参考图片Western blot analysis of extracts from HeLa and Jurkat cells using BLM Antibody.使用BLM抗体利用western blot方法检测HeLa细胞和Jurkat细胞提取物。 |
