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蛋白质/抗原/多肽 >> 重组蛋白
重组Dysfip1蛋白
重组Dysfip1蛋白图片
交货期: 1周
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交货期: 1周
半岛bd体育手机客户端 别名: Recombinant Ppp1r27
Recombinant protein phosphatase 1, regulatory subunit 27 protein
半岛bd体育手机客户端 介绍
基因名:

Ppp1r27


半岛bd体育手机客户端 别名:

1110033I14Rik; Dysfip1; toonin; Ppp1r27; protein phosphatase 1, regulatory subunit 27; protein phosphatase 1, regulatory subunit 27; protein phosphatase 1 regulatory subunit 27; dysferlin-interacting protein 1; ortholog of human dysferlin interacting protein 1 (toonin) DYSFP1; Dysfip1蛋白;


背景信息:
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, adult-onset, distal dystrophy known as Miyoshi myopathy (MM) (1,2). Dysferlin localizes to the muscle fiber membrane, but 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 adulthood (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).

标签:His-tag
分类:Recombinant
类型:Protein
偶联物:Unconjugated
内毒素水平:按批次,参阅瓶身标签
性状:Liquid
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
纯化类型:purified
内含物:不含防腐剂
应用:Positive Control;Immunogen;SDS-PAGE;WB.
储存:2-8°C不超过一个月,-80°C不超过12个月。避免反复冻融。
注意事项:仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。

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