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Rabbit Monoclonal deltaSarcoglycan Antibody

  • 中文名: deltaSarcoglycan抗体
  • 别    名: 35DAG; CMD1L; DAGD; Delta-sarcoglycan; LGMD2F; SG delta; SGCD; SGCDP; ;delta Sarcoglycan
货号: IPDX17630
Price: ¥1280
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 1/20-1/50 Human,Mouse,Rat
IHC 1/100-1/200 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

Aliases35DAG; CMD1L; DAGD; Delta-sarcoglycan; LGMD2F; SG delta; SGCD; SGCDP; ;delta Sarcoglycan
WB Predicted band sizeCalculated MW: 32 kDa ; Observed MW: 35 kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman
ImmunogenA synthesized peptide derived from human delta Sarcoglycan
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

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参考文献

以下是3篇关于δ-Sarcoglycan抗体的参考文献,按文献名称、作者和摘要内容简要概括:

1. **文献名称**:Mutations in the δ-sarcoglycan gene are a rare cause of autosomal recessive limb-girdle muscular dystrophy

**作者**:Bönnemann CG, et al.

**摘要**:该研究通过免疫组化分析发现δ-sarcoglycan抗体可用于检测肌肉活检样本中蛋白表达缺失,确认δ-SG基因突变导致肢带型肌营养不良(LGMD2F),并讨论了抗体在疾病分型中的诊断价值。

2. **文献名称**:Differential expression of sarcoglycan isoforms in skeletal muscle of patients with limb-girdle muscular dystrophy

**作者**:Jung D, et al.

**摘要**:研究利用δ-sarcoglycan特异性抗体比较健康人与LGMD患者的肌肉组织,发现δ-SG蛋白在患者中显著减少或异常聚集,提示其作为生物标志物在肌肉病理评估中的应用潜力。

3. **文献名称**:Immunohistochemical analysis of sarcoglycans in human cardiomyopathies

**作者**:Kawada T, et al.

**摘要**:通过δ-sarcoglycan抗体检测扩张型心肌病患者心肌组织,发现δ-SG表达异常与心肌细胞膜稳定性下降相关,揭示了其在维持心脏结构完整性中的关键作用。

背景信息

Delta-sarcoglycan, a key component of the sarcoglycan complex, is a transmembrane protein critical for maintaining the structural integrity of muscle cell membranes. This complex, which includes alpha-, beta-, gamma-, and delta-sarcoglycan subunits, interacts with dystrophin and other proteins to form the dystrophin-glycoprotein complex (DGC). The DGC stabilizes muscle fibers during contraction by linking the cytoskeleton to the extracellular matrix, protecting cells from mechanical stress. Mutations in the delta-sarcoglycan gene (SGCD) disrupt this complex, leading to limb-girdle muscular dystrophy type 2F (LGMD2F), a progressive disorder characterized by muscle weakness and degeneration.

Antibodies targeting delta-sarcoglycan are essential tools in research and diagnostics. They enable the detection and localization of delta-sarcoglycan in tissues, helping to assess protein expression levels in muscular dystrophy models or patient biopsies. These antibodies are widely used in techniques like immunohistochemistry, Western blotting, and immunofluorescence to study disease mechanisms, validate gene therapy outcomes, or confirm SGCD mutations. Commercial delta-sarcoglycan antibodies are typically raised in rabbits or mice, often validated for specificity against recombinant or endogenous protein.

Understanding delta-sarcoglycan’s role and its associated antibodies advances both the diagnosis of LGMD2F and the development of targeted therapies, such as gene replacement or protein stabilization strategies.

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