gamma Sarcoglycan Recombinant Rabbit Monoclonal Antibody [JA11-57]
Catalog# ET1704-24
gamma Sarcoglycan Recombinant Rabbit Monoclonal Antibody [JA11-57]
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WB
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IP
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IHC-P
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Human
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Mouse
Predicted species support after-sales service
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unconjugated
Overview
Product Name
gamma Sarcoglycan Recombinant Rabbit Monoclonal Antibody [JA11-57]
Antibody Type
Recombinant Rabbit monoclonal Antibody
Immunogen
Recombinant protein within Human gamma Sarcoglycan aa 42-291 / 291.
Species Reactivity
Human (Predicted: Mouse)
Predicted species support after-sales service
Validated Applications
WB, IP, IHC-P
Molecular Weight
Predicted band size: 32 kDa
Positive Control
Human skeletal muscle tissue lysates, human striated muscle tissue.
Conjugation
unconjugated
Clone Number
JA11-57
RRID
Product Features
Form
Liquid
Concentration
Storage Instructions
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). It is recommended to aliquot into single-use upon delivery. Store at -20℃ long term.
Storage Buffer
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Isotype
IgG
Purification Method
Protein A affinity purified.
Application Dilution
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WB
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1:500-1:2,000
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IHC-P
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1:50-1:200
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IP
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Use at an assay dependent concentration.
Target
Function
The sarcoglycan transmembrane proteins are members of the dystrophin complex. Sarcoglycans cluster together to form a complex, which is localized in the cell membrane of skeletal, cardiac and smooth muscle fibers. Four sarcoglycan subunit proteins, designated α-, β-, γ- and δ-sarcoglycan, form a complex on the skeletal muscle cell surface membrane. A genetic defect in any one of these proteins causes the loss or marked decrease of the whole sarcoglycan complex, which is observed in the autosomal recessive muscular dystrophy, sarcoglycanopathy. In smooth muscle, β- and δ-sarcoglycans are associated with ε-sarcoglycan, a glycoprotein homologous to α-sarcoglycan. Additionally, a complete deficiency in δ-sarcoglycan is the cause of the Syrian hamster BIO.14 cardiomyopathy.
Background References
1. Van den Bergen JC et al. Clinical characterisation of Becker muscular dystrophy patients predicts favourable outcome in exon-skipping therapy. J Neurol Neurosurg Psychiatry N/A:N/A (2013).
2. Cingolani, E. et al. 2014. Engineered Electrical Conduction Tract Restores Conduction in Complete Heart Block J Am Coll Cardiol.. 64: 2575-2585.
Sequence Similarity
Belongs to the sarcoglycan beta/delta/gamma/zeta family.
Tissue Specificity
Expressed in skeletal and heart muscle.
Subcellular Location
Cell membrane. Cytoplasm.
UNIPROT
Synonyms
35 kDa dystrophin associated glycoprotein antibody
35 kDa dystrophin-associated glycoprotein antibody
35DAG antibody
35kD dystrophin associated glycoprotein antibody
35kDa dystrophin-associated glycoprotein antibody
A4 antibody
DAGA4 antibody
DMDA antibody
DMDA1 antibody
Gamma SG antibody
ExpandImages
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Western blot analysis of gamma Sarcoglycan on human skeletal muscle tissue lysates. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (ET1704-24, 1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1:5,000 dilution was used for 1 hour at room temperature.
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Immunohistochemical analysis of paraffin-embedded human striated muscle tissue with Rabbit anti-gamma Sarcoglycan antibody (ET1704-24) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1704-24) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"