Recombinant Human alpha Sarcoglycan protein (ab114733)

製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号Q16586
    • 生物種Human
    • 配列TTLHPLVGRVFVHTLDHETFLSLPEHVAVPPAVHITYHAHLQGHPDLPRW LRYTQRSPHHPGFLYGSATPEDRGLQVIEVTAYNRDSFDTTRQRLVLEIG DPEGPLLP
    • 分子量38 kDa including tags
    • 領域26 to 133

特性

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.

  • アプリケーション

    ELISA

    Western blot

    SDS-PAGE

  • 製品の状態Liquid
  • 備考Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能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.
  • 組織特異性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.
  • 関連疾患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.
  • 配列類似性Belongs to the sarcoglycan alpha/epsilon family.
  • 細胞内局在Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
  • Information by UniProt

Recombinant Human alpha Sarcoglycan protein 画像

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

Recombinant Human alpha Sarcoglycan protein (ab114733) 使用論文

ab114733 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab114733.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"