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产品中心 药物靶点和Fc受体 药物靶点

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Plectin His Tag Protein, Human

PCN, PLTN,Plectin-1,PLEC1

价格 ￥1,390.00 （ 供应商现货 ： 3-5个工作日）
货号 UA016100
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产品介绍

产品介绍 评论(0)

产品规格

  • 物种

    Human

  • 分子别名

    PCN, PLTN, Plectin-1, PLEC1

  • Accession

    Q15149-1

  • 表达序列

    Ala4377-Ala4684 with His Tag at the N-Terminus

  • 表达宿主

    HEK293

  • 分子量

    40-60kDa (Reducing)

  • 纯度

    ＞90% by SDS-PAGE

  • 标记

    Unconjugated

  • 标签

    His Tag

  • 性状

    Lyophilized Powder

  • 缓冲体系

    PBS, pH7.4, 5% trehalose

  • 溶解方法

    Reconstitute at 0.1-1 mg/ml according to the size in ultrapure water after rapid centrifugation.

  • 储存条件

    · 12 months from date of receipt, lyophilized powder stored at -20 to -80℃.
    · 3 months, -20 to -80℃ under sterile conditions after reconstitution.
    · 1 week, 2 to 8℃ under sterile conditions after reconstitution.
    · Please avoid repeated freeze-thaw cycles.

  • 文献引用

    1.Smith FJ, Eady RA, Leigh IM, McMillan JR, Rugg EL, Kelsell DP, Bryant SP, Spurr NK, Geddes JF, Kirtschig G, Milana G, de Bono AG, Owaribe K, Wiche G, Pulkkinen L, Uitto J, McLean WH, Lane EB. Plectin deficiency results in muscular dystrophy with epidermolysis bullosa. Nat Genet. 1996 Aug;13(4):450-7.
    2.McLean WH, Pulkkinen L, Smith FJ, Rugg EL, Lane EB, Bullrich F, Burgeson RE, Amano S, Hudson DL, Owaribe K, McGrath JA, McMillan JR, Eady RA, Leigh IM, Christiano AM, Uitto J. Loss of plectin causes epidermolysis bullosa with muscular dystrophy: cDNA cloning and genomic organization. Genes Dev. 1996 Jul 15;10(14):1724-35.

背景介绍

  • Plectin is a large cytolinker protein composed of an N-terminal actin-binding domain, a central rod-shaped α-helical coiled-coil region, and a C-terminal intermediate filament-binding domain, with multiple alternatively spliced isoforms enabling diverse subcellular localization; its functional core lies in bridging microfilaments, intermediate filaments, and microtubule networks to maintain cellular mechanical stability, while also participating in hemidesmosome assembly, myofibril integrity maintenance, and signal transduction regulation. Clinically, PLEC gene mutations cause epidermolysis bullosa simplex (EBS) and its variant subtypes with muscular dystrophy, pyloric atresia, or cardiomyopathy, collectively termed "plectinopathies," with pathological mechanisms involving dermal-epidermal junction disruption, desmin aggregation in muscle fibers, and mitochondrial dysfunction, while recent therapeutic strategies such as chemical chaperone approaches have provided new directions for treatment.

  • 电泳

    • 1μg (R: reducing condition).

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