- 产品信息
商品描述
靶点-Target
FKRP
反应性
Human,Mouse,Rat
应用
WB,IF,ELISA
宿主物种-Host Species
Rabbit
同种型-Isotype
IgG
修饰-Modification
Unmodified
MW(Observed)
50kD
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
免疫原
The antiserum was produced against synthesized peptide derived from human FKRP. AA range:1-50
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
Organism-1
Human
基因ID-1
79147
SwissProt-1
Q9H9S5
Organism-2
Mouse
基因ID-2
243853
SwissProt-2
Q8CG64
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). .





