Dysferlin Polyclonal Antibody

产品详情

产品名称

Dysferlin Polyclonal Antibody

产品货号

YM-A18018

规格

50μL;100μL

种属反应

Human,Mouse

实验应用

WB,IF,ELISA

分子量

240kD

宿主

Rabbit

同种型

IgG

修饰

Unmodified

推荐稀释比

WB 1:500-1:2000;IF 1:200-1:1000;ELISA 1:10000;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

克降性

Polyclonal

克隆号

免疫原

The antiserum was produced against synthesized peptide derived from human Dysferlin. AA range:1981-2030

特异性

Dysferlin Polyclonal Antibody detects endogenous levels of Dysferlin protein.

基因名称

DYSF

蛋白名称

Dysferlin

别名

DYSF;FER1L1;Dysferlin;Dystrophy-associated fer-1-like protein;Fer-1-like protein 1

基因ID-1

8291

SwissProt

O75923

背景

dysferlin(DYSF) Homo sapiens The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008],

细胞定位

Cell membrane, sarcolemma; Single-pass type II membrane protein. Cytoplasmic vesicle membrane ; Single-pass type II membrane protein . Cell membrane. Colocalizes, during muscle differentiation, with BIN1 in the T-tubule system of myotubules and at the site of contact between two myotubes or a myoblast and a myotube. Wounding of myotubes led to its focal enrichment to the site of injury and to its relocalization in a Ca(2+)-dependent manner toward the plasma membrane. Colocalizes with AHNAK, AHNAK2 and PARVB at the sarcolemma of skeletal muscle. Detected on the apical plasma membrane of the syncytiotrophoblast. Reaches the plasmma membrane through a caveolin-independent mechanism. Retained by caveolin at the plasmma membrane (By similarity). Colocalizes, during muscle differentiation, with CACNA1S in the T-tubule system of myotubules (By similarity). Accumulates and colocalizes with fusion vesicles at the sarcolemma disruption sites (By similarity). .