Fukutin-related protein (FKRP) is targeted to the medial Golgi appaRatus and is necessary for posttranslational modification of dystroglycan. Mutations in this gene have been associated with congenital muscular dystrophy, mental retardation, and cerebellar cysts. Mutations in fukutin-related protein (FKRP) give rise to mild and more severe forms of muscular dystrophy. FKRP may influence the glycosylation status of dystroglycan from within the sarcolemmal dystrophin-glycoprotein complex. FKRP associates with the sarcolemmal DGC and that it plays a unique role in dystroglycanopathy muscle disease.
应用类型
Enzyme-linked immunosorbent assay|Flow cytometry|Immunohistochemistry|Immunoprecipitation|Western blot
免疫原
A synthetic peptide corresponding to the C-terminus of human FKRP protein. This sequence is identical to human, mouse and rat
来源宿主
Rabbit
反应性
该Anti-FKRP Polyclonal 抗体的反应性请参考该产品的说明书
保存建议
The antibodies are stable for 24 months from date of receipt when stored at -20C to -70C. The antibodies can be stored at 2C-8C for three month without detectable loss of activity. Avoid repeated freezing-thawing cycles.
其他
Aviva Systems Biology总部位于加利福尼亚州圣迭戈，在中国北京设有办公室，专注于为研究需求提供多克隆和单克隆抗体、ELISA试剂盒、蛋白质和定制服务。Aviva Systems Biology生产了24,000种经过验证的多克隆抗体，并提供近20,000种ELISA试剂盒，定制实验室服务包括蛋白表达和纯化、抗体开发，以及ELISA的开发、验证和生产。Aviva Systems Biology为与独特物种和靶标相关的研究提供独特工具，研究领域包括转录因子、癌症、心血管、细胞生物学、DNA损伤和修复、表观遗传学、信号转导、细胞分化、干细胞生物学等等。
