Anti-Dysferlin (N-terminal) antibody produced in rabbit

半岛bd体育手机客户端 说明

一般描述

Dysferlin is a 230 kDa transmembrane protein that belongs to the ferlin family of proteins including myoferlin and otoferlin and is homologous to theC. elegansfer-1 protein. It is expressed early during human development.

免疫原

synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin, conjugated to KLH. The corresponding sequence is identical in human dysferlin isoforms 1-14 and identical in mouse dysferlin.

应用

Anti-Dysferlin (N-terminal) antibody produced in rabbit has been used in several immunochemical techniques including immunoblotting, immunofluorescence and immunohistochemistry.

生化/生理作用

Dysferlin is implicated in membrane fusion events. It has also been involved in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. The integral membrane protein caveolin 3 have been shown to regulate the endocytosis of dysferlin. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and 3, resulting in mistargeting and redistribution of dysferlin from the plasma membrane to the Golgi complex. Mutations in the dysferlin gene leads to the development of limb-girdle muscle dystrophy type 2B (LGMD2B), an autosomal recessive disorder and the related Miyoshi myopathy.

外形

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

基本信息

NACRES

NA.41

半岛bd体育手机客户端 性质

生物来源	rabbit
质量水平	200
偶联物	unconjugated
抗体形式	affinity isolated antibody
antibody product type	primary antibodies
克隆	polyclonal
形式	buffered aqueous solution
分子量	antigen ~250 kDa
species reactivity	rat, human, mouse
增强验证	recombinant expression Learn more aboutAntibody Enhanced Validation
浓度	~1.0 mg/mL
technique(s)	dot blot: 0.1-0.2 μg/mL using extracts of HEK-293T cells over-expressing human dysferlin and 1-2 mg/mL using A10 cells. immunohistochemistry: 20-30 μg/mL using methanol-acetone fixed frozen sections of mouse skeletal muscle. indirect immunofluorescence: 5-10 μg/mL using differentiated C2C12 myoblasts.
UniProt登记号	O75923
运输	dry ice
储存温度	?20℃
Gene Information	human ... DYSF(8291) mouse ... Dysf(26903) rat ... Dysf(312492)

安全信息

储存分类代码	12 - Non Combustible Liquids
闪点(F)	Not applicable
闪点(C)	Not applicable