Recombinant Human alpha Sarcoglycan protein (ab132339)
- Datasheet
- References
- Protocols
Overview
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Product name
Recombinant Human alpha Sarcoglycan protein -
Protein length
Full length protein
Description
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Nature
Recombinant -
Source
Wheat germ -
Amino Acid Sequence
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Accession
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Species
Human -
Sequence
MAETLFWTPLLVVLLAGLGDTEAQQTTLHPLVGRVFVHTLDHETFLSLPE HVAVPPAVHITYHAHLQGHPDLPRWLRYTQRSPHHPGFLYGSATPEDRGL QVIEVTAYNRDSFDTTRQRLVLEIGDPEGPLLPYQAEFLVRSHDAEEVLP STPASRFLSALGGLWEPGELQLLNVTSALDRGGRVPLPIEGRKEGVYIKV GSASPFSTCLKMVASPDSHARCAQGQPPLLSCYDTLAPHFRVDWCNVTLV DKSVPEPADEVPTPGDGILEHDPFFCPPTEAPDRDFLVDALVTLLVPLLV ALLLTLLLAYVMCCRREGRLKRDLATSDIQMVHHCTIHGNTEELRQMAAS REVPRPLSTLPMFNVHTGERLPPRVDSAQVPLILDQH -
Molecular weight
69 kDa including tags -
Amino acids
1 to 387
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Specifications
Our Abpromise guarantee covers the use of ab132339 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
ELISA
Western blot
SDS-PAGE
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Form
Liquid -
Additional notes
Protein 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
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Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.31% Glutathione, 0.79% Tris HCl
General Info
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Alternative names
- 50 DAG
- 50 kDa dystrophin associated glycoprotein
- 50 kDa dystrophin-associated glycoprotein
see all -
Function
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. -
Tissue specificity
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. -
Involvement in disease
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. -
Sequence similarities
Belongs to the sarcoglycan alpha/epsilon family. -
Cellular localization
Cell membrane > sarcolemma. Cytoplasm > cytoskeleton. - Information by UniProt
Images
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SDS-PAGE - Recombinant Human alpha Sarcoglycan protein (ab132339)12.5% SDS-PAGE analysis of ab132339 stained with Coomassie Blue.
Datasheets and documents
References
ab132339 has not yet been referenced specifically in any publications.
