产品详情
简单介绍：
Anti-Dysferlin interacting protein 1抗体产品质量稳定，实验效果明显，货期快，价格优惠，欢迎垂询订购！我公司长期供应**组化抗体、WB抗体、**组化试剂盒和抗体试验所需全部相关试剂、荧光标记抗体、单克隆抗体、多克隆抗体、各种标记的二抗IgG/IgM/IgD/IgA等科研实验抗体。Anti-Dysferlin interacting protein 1抗体用于**组化实验,WB实验,相应的标记抗体有HRP标记抗体,FITC标记,BIO等。
详情介绍：
Rabbit Anti-Dysferlin interacting protein 1
Cat. Number:
Anti-Dysferlin interacting protein 1抗体KL-14476R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Dysferlin is a muscle-specific protein that is essential for normal muscle function and development (1). Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.3-p13.1, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, *****-onset, distal dystrophy known as Miyoshi myopathy (MM) (1,2). Dysferlin localizes to the muscle fiber membrane, but
Anti-Dysferlin interacting protein 1抗体is absent in MM and LGMD-2B muscle (1,3). Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation (3). Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle (1). It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in *****hood (3). Identical mutations in the dysferlin gene can produce more than one myopathy phenotype, indicating that additional genes and/or other factors are also involved in the clinical phenotype (4,5). The DYSF gene has no homology to any other known mammalian gene, but the protein product is related to the spermatogenesis factor fer-1 of Caenorhabditis elegans. The name ‘dysferlin’ combines the role of the gene in producing muscular dystrophy with its homology to C. elegans (6).
Also known as:
dysferlin interacting protein 1 (toonin);
Anti-Dysferlin interacting protein 1抗体Dysfip1; PPR27_HUMAN; Toonin.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Dog, Cow, Horse, Sheep, .
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Immunogen: KLH conjugated synthetic peptide derived from human Dysferli.
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Predicted Molecular Weight: 17kDa.
Storage:
Shipped at 4℃, Store at -20℃
Anti-Dysferlin interacting protein 1抗体(Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
Not yet tested in other applications. Anti-Dysferlin interacting protein 1抗体
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications. Anti-Dysferlin interacting protein 1抗体
Optimal working dilutions must be determined by the end user.
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