Recombinant人alpha Sarcoglycan protein (ab114733)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionQ16586
    • 种属Human
    • 序列TTLHPLVGRVFVHTLDHETFLSLPEHVAVPPAVHITYHAHLQGHPDLPRW LRYTQRSPHHPGFLYGSATPEDRGLQVIEVTAYNRDSFDTTRQRLVLEIG DPEGPLLP
    • 分子量38 kDa including tags
    • 氨基酸26 to 133

技术指标

Our Abpromise guarantee covers the use of ab114733 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    ELISA

    Western blot

    SDS-PAGE

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • 50 DAG
    • 50 kDa dystrophin associated glycoprotein
    • 50 kDa dystrophin-associated glycoprotein
    • 50DAG
    • 50kD DAG
    • 59kDa
    • A2
    • adhalin
    • ADL
    • Alpha SG
    • Alpha-sarcoglycan
    • Alpha-SG
    • Asg
    • DAG2
    • DMDA2
    • Dystroglycan 2
    • Dystroglycan-2
    • LGMD2D
    • sarcoglycan, alpha (dystrophin-associated glycoprotein)
    • SCARMD1
    • Sgca
    • SGCA_HUMAN
    see all
  • 功能Component of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.
  • 组织特异性Most strongly expressed in skeletal muscle. Also expressed in cardiac muscle and, at much lower levels, in lung. In the fetus, most abundant in cardiac muscle and, at lower levels, in lung. Also detected in liver and kidney. Not expressed in brain.
  • 疾病相关Defects in SGCA are the cause of limb-girdle muscular dystrophy type 2D (LGMD2D) [MIM:608099]; also known as Duchenne-like muscular dystrophy autosomal recessive type 2 or severe childhood autosomal recessive muscular dystrophy (SCARMD). LGMD2D is an autosomal recessive degenerative myopathy characterized by progressive muscle wasting from early childhood with loss of independent ambulation by teenage years. Muscle biopsy shows necrosis, decreased immunostaining for alpha sarcoglycan, and adhalin deficiency. The phenotype is less severe than LGMD2C.
  • 序列相似性Belongs to the sarcoglycan alpha/epsilon family.
  • 细胞定位Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
  • Information by UniProt

Recombinant Human alpha Sarcoglycan protein 图像

  • 12.5% SDS-PAGE showing ab114733 at approximately 37.51kDa.
    Stained with Coomassie Blue.

Recombinant Human alpha Sarcoglycan protein (ab114733)参考文献

ab114733 has not yet been referenced specifically in any publications.

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