SGCB 抗体 (AA 101-318)
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北京 101111
Quick Overview for SGCB 抗体 (AA 101-318) (ABIN7869898)
抗原
See all SGCB 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 101-318
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原理
- SGCB Antibody / Beta Sarcoglycan
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纯化方法
- Immunogen affinity purified
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免疫原
- E.coli-derived human Beta Sarcoglycan/SGCB recombinant protein (Position: E101-H318) was used as the immunogen for the SGCB antibody.
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亚型
- IgG
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应用备注
- Optimal dilution of the SGCB antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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储存条件
- 4 °C,-20 °C
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储存方法
- After reconstitution, the SGCB antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
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- SGCB (Sarcoglycan, beta (43kDa Dystrophin-Associated Glycoprotein) (SGCB))
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别名
- SGCB
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背景
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SGCB antibody recognizes Beta sarcoglycan, a transmembrane glycoprotein that forms part of the dystrophin-associated glycoprotein complex (DGC) in muscle cells. This complex stabilizes the sarcolemma and links the cytoskeleton to the extracellular matrix. The SGCB gene encodes the beta subunit of the sarcoglycan complex, which also includes alpha, gamma, and delta sarcoglycans. Mutations in SGCB cause limb-girdle muscular dystrophy type 2E (LGMD2E), characterized by progressive muscle weakness and degeneration. The SGCB antibody is used extensively in muscular dystrophy research, enabling analysis of sarcoglycan expression, localization, and pathological loss in patient samples and model systems.
Beta sarcoglycan is a 318-amino acid membrane protein encoded on human chromosome 4q12. It contains a large extracellular domain with multiple N-glycosylation sites, a single transmembrane segment, and a short cytoplasmic tail. Proper assembly of the sarcoglycan complex is essential for maintaining membrane integrity during muscle contraction. Loss or misfolding of any component can destabilize the complex and lead to muscle fiber damage. Immunohistochemistry with SGCB antibody reveals strong sarcolemmal staining in normal skeletal and cardiac muscle, whereas dystrophic samples often show complete or partial absence of the protein.
The SGCB antibody is valuable for diagnostic and mechanistic studies of muscular dystrophies. In western blot, beta sarcoglycan is typically detected at 43i 1/245 kDa. Its expression patterns provide insights into disease progression, compensatory mechanisms, and the effectiveness of gene replacement therapies. Research has shown that restoring SGCB expression in animal models can reassemble the DGC and improve muscle function, highlighting its potential as a therapeutic target. Moreover, beta sarcoglycan plays roles in cardiomyocyte adhesion and signal transduction, extending its relevance beyond skeletal muscle.
NSJ Bioreagents supplies validated SGCB antibodies compatible with immunohistochemistry, immunofluorescence, and western blotting, ensuring reliable detection in human and animal tissues. These reagents support ongoing research into sarcolemmal stability, muscular dystrophy pathogenesis, and therapeutic intervention strategies that aim to restore DGC integrity and muscle resilience. -
UniProt
- Q16585
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途径
- Myometrial Relaxation and Contraction, Maintenance of Protein Location
抗原
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