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货号: PD5728
货期: 现货
价格: 50μL/960;100μL/1600;200μL/2560
所属分类：
靶点: FKRP
应用： WB,IF,ELISA
反应性 ： Human,Mouse,Rat
宿主物种 ： Rabbit
MW(Observed)： 50kD
同种型-Isotype ： IgG
隐藏域元素占位
详细信息
推荐稀释比 ： WB 1:500-1:2000;IF 1:200-1:1000;ELISA 1:20000;Not yet tested in other applications.
组成 ： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺 ： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
储存 ： -15°C to -25°C/1 year(Do not lower than -25°C
浓度 ： 1 mg/ml
克隆性 ： Polyclonal
抗原&靶点信息
特异性 ： FKRP Polyclonal Antibody detects endogenous levels of FKRP protein.
基因名称 ： FKRP
蛋白名称 ： Fukutin-related protein
别名 ： FKRP;Fukutin-related protein
背景：
This gene encodes a protein which 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. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq, Oct 2008],
细胞定位 ： Golgi apparatus membrane ; Single-pass type II membrane protein . Secreted . Cell membrane, sarcolemma . Rough endoplasmic reticulum . Cytoplasm . According to some studies the N-terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted (PubMed:19900540). Localization at the cell membrane may require the presence of dystroglycan (By similarity). At the Golgi apparatus localizes to the middle-to-trans-cisternae, as assessed by MG160 colocalization. Detected in rough endoplasmic reticulum in myocytes (PubMed:17554798, PubMed:21886772). In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum (PubMed:15213246). .
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