Recombinant Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)
![Western blot - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)](https://www.abcam.com/ps/products/189/ab189254/Images/ab189254-224585-anti-alpha-sarcoglycan-antibody-epr14773-western-blot.jpg "Western blot - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)")
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR14773] to alpha Sarcoglycan
- Suitable for: IHC-P, WB, IP
- Reacts with: Mouse, Rat, Human
Related conjugates and formulations
Overview
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Product name
Anti-alpha Sarcoglycan antibody [EPR14773]
See all alpha Sarcoglycan primary antibodies -
Description
Rabbit monoclonal [EPR14773] to alpha Sarcoglycan -
Host species
Rabbit -
Tested applications
Suitable for: IHC-P, WB, IPmore details -
Species reactivity
Reacts with: Mouse, Rat, Human -
Immunogen
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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Positive control
- Human skeletal muscle, mouse heart and rat heart lysates; Human skeletal muscle tissue.
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General notes
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.2
Preservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA -
Concentration information loading... -
Purity
Protein A purified -
Clonality
Monoclonal -
Clone number
EPR14773 -
Isotype
IgG -
Research areas
Associated products
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Alternative Versions
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Isotype control
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Positive Controls
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Recombinant Protein
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Related Products
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab189254 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| IHC-P |
1/1000 - 1/2000. Perform heat mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.
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| WB |
1/1000 - 1/10000. Detects a band of approximately 50 kDa (predicted molecular weight: 43 kDa).
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| IP |
1/30.
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| Notes |
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IHC-P
1/1000 - 1/2000. Perform heat mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol. |
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WB
1/1000 - 1/10000. Detects a band of approximately 50 kDa (predicted molecular weight: 43 kDa). |
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IP
1/30. |
Target
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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
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Database links
- Entrez Gene: 6442 Human
- Entrez Gene: 20391 Mouse
- Entrez Gene: 303468 Rat
- Omim: 600119 Human
- SwissProt: Q16586 Human
- SwissProt: P82350 Mouse
- SwissProt: Q5SWB2 Mouse
- Unigene: 463412 Human
see all -
Alternative names
- 50 DAG antibody
- 50 kDa dystrophin associated glycoprotein antibody
- 50 kDa dystrophin-associated glycoprotein antibody
see all
Images
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Western blot - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254) at 1/10000 dilution + Human skeletal muscle lysate at 20 µg
Secondary
Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugate at 1/1000 dilution
Predicted band size: 43 kDa
Observed band size: 50 kDa why is the actual band size different from the predicted? -
![Western blot - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)](https://www.abcam.com/ps/products/189/ab189254/Images/ab189254-224584-anti-alpha-sarcoglycan-antibody-epr14773-western-blot.jpg)
Western blot - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)All lanes : Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254) at 1/1000 dilution
Lane 1 : Mouse heart lysate
Lane 2 : Rat heart lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugate at 1/1000 dilution
Predicted band size: 43 kDa
Observed band size: 50 kDa why is the actual band size different from the predicted? -
![Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)](https://www.abcam.com/ps/products/189/ab189254/Images/ab189254-224583-anti-alpha-sarcoglycan-antibody-epr14773-immunohistochemistry.jpg)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)Immunhistochemical analysis of paraffin-embedded Human skeletal muscle tissue labeling alpha Sarcoglycan with ab189254 at 1/2000 dilution, followed by prediluted HRP Polymer for Rabbit IgG. Counter stained with Hematoxylin.
Perform heat mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.
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![Immunoprecipitation - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)](https://www.abcam.com/ps/products/189/ab189254/Images/ab189254-224582-anti-alpha-sarcoglycan-antibody-epr14773-immunoprecipitation.jpg)
Immunoprecipitation - Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)Immunoprecipitation analysis of human skeletal muscle tissue lysate labeling alpha Sarcoglycan using ab189254 at 1/50 dilution (Lane 1). A Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1500 was used as secondary antibody. Lane 2: PBS instead of human skeletal muscle tissue lysate.
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![Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)](https://www.abcam.com/ps/products/189/ab189254/Images/ab189254-5-benefits-of-recombinant-antibodies.png)
Anti-alpha Sarcoglycan antibody [EPR14773] (ab189254)
Protocols
Datasheets and documents
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SDS download
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Datasheet download
Certificate of Compliance
References (7)
ab189254 has been referenced in 7 publications.
- Griffin DA et al. Preclinical Systemic Delivery of Adeno-Associated a-Sarcoglycan Gene Transfer for Limb-Girdle Muscular Dystrophy. Hum Gene Ther 32:390-404 (2021). PubMed: 33349138
- Fukuoka M et al. MiR-199-3p enhances muscle regeneration and ameliorates aged muscle and muscular dystrophy. Commun Biol 4:427 (2021). PubMed: 33782502
- Carotti M et al. Combined Use of CFTR Correctors in LGMD2D Myotubes Improves Sarcoglycan Complex Recovery. Int J Mol Sci 21:N/A (2020). PubMed: 32155735
- Gerli MFM et al. Combined Notch and PDGF Signaling Enhances Migration and Expression of Stem Cell Markers while Inducing Perivascular Cell Features in Muscle Satellite Cells. Stem Cell Reports 12:461-473 (2019). PubMed: 30745033
- Egorova TV et al. CRISPR/Cas9-generated mouse model of Duchenne muscular dystrophy recapitulating a newly identified large 430 kb deletion in the human DMD gene. Dis Model Mech 12:N/A (2019). PubMed: 31028078
- Zhao K et al. Sarcoglycan Alpha Mitigates Neuromuscular Junction Decline in Aged Mice by Stabilizing LRP4. J Neurosci 38:8860-8873 (2018). PubMed: 30171091
- Carotti M et al. Repairing folding-defective a-sarcoglycan mutants by CFTR correctors, a potential therapy for limb-girdle muscular dystrophy 2D. Hum Mol Genet 27:969-984 (2018). PubMed: 29351619