Recombinant Human alpha Sarcoglycan protein (ab114733)

Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionQ16586
    • SpeciesHuman
    • SequenceTTLHPLVGRVFVHTLDHETFLSLPEHVAVPPAVHITYHAHLQGHPDLPRW LRYTQRSPHHPGFLYGSATPEDRGLQVIEVTAYNRDSFDTTRQRLVLEIG DPEGPLLP
    • Molecular weight38 kDa including tags
    • Amino acids26 to 133

Specifications

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.

  • Applications

    ELISA

    Western blot

    SDS-PAGE

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names
    • 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
  • FunctionComponent of the sarcoglycan complex, a subcomplex of the dystrophin-glycoprotein complex which forms a link between the F-actin cytoskeleton and the extracellular matrix.
  • Tissue specificityMost 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.
  • Involvement in diseaseDefects 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.
  • Sequence similaritiesBelongs to the sarcoglycan alpha/epsilon family.
  • Cellular localizationCell membrane > sarcolemma. Cytoplasm > cytoskeleton.
  • Information by UniProt

Recombinant Human alpha Sarcoglycan protein images

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

References for Recombinant Human alpha Sarcoglycan protein (ab114733)

ab114733 has not yet been referenced specifically in any publications.

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