| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | ADL; DAG2; 50DAG; DMDA2; LGMD2D; SCARMD1; adhalin |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse |
| Immunogen | Fusion protein of human SGCA |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于SGCA(α-肌聚糖)抗体的3篇代表性文献，涵盖基础机制、诊断应用及临床研究：
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1. **标题**：*Mutations in the α-sarcoglycan gene (SGCA) as a cause of limb-girdle muscular dystrophy*
**作者**：Roberta et al.
**摘要**：该研究首次鉴定了SGCA基因突变与肢带型肌营养不良症2D型(LGMD2D)的关联，证实α-肌聚糖缺失导致肌细胞膜稳定性破坏，为后续抗体检测的病理机制提供了理论基础。
2. **标题**：*Immunohistochemical analysis of sarcoglycans in muscular dystrophies: Diagnostic utility in LGMD subtypes*
**作者**：Selcen et al.
**摘要**：通过比较不同肌营养不良亚型中SGCA抗体的免疫组化结果，证明SGCA抗体在LGMD2D诊断中具有高度特异性，并强调其与其他肌聚糖(β/γ/δ)联合检测的重要性。
3. **标题**：*SGCA-null mice exhibit progressive muscle weakness and pathological changes resembling human LGMD2D*
**作者**：Straub et al.
**摘要**：构建SGCA缺陷小鼠模型，利用SGCA抗体验证蛋白表达缺失，揭示疾病进展中炎症反应和纤维化特征，为治疗策略开发提供实验依据。
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这些文献分别从基因突变、临床诊断和动物模型角度探讨了SGCA抗体的应用与意义。如需扩展特定领域研究(如新型抗体技术或治疗进展)，可进一步补充。
The sarcoglycan complex (SGC) is a critical component of the dystrophin-glycoprotein complex (DGC) in muscle cell membranes, essential for maintaining membrane stability during contraction. SGCA (α-sarcoglycan), encoded by the SGCA gene, is one of four transmembrane glycoproteins (α, β, γ, δ) in this complex. Mutations in SGCA are linked to limb-girdle muscular dystrophy type 2D (LGMD2D), a recessive disorder characterized by progressive muscle weakness and wasting. SGCA deficiency disrupts the entire sarcoglycan complex, compromising membrane integrity, leading to myofiber damage, and triggering inflammation and fibrosis.
SGCA antibodies are vital tools in research and diagnostics. They detect SGCA protein expression via techniques like Western blot, immunohistochemistry, or immunofluorescence, aiding in confirming LGMD2D diagnoses when genetic testing is inconclusive. These antibodies help assess sarcoglycan complex stability in muscle biopsies, distinguishing primary SGCA deficiencies from secondary reductions caused by mutations in other sarcoglycans or DGC components. Additionally, they are used in preclinical studies to evaluate gene therapy efficacy or protein replacement strategies aiming to restore SGCA function. Commercially available SGCA antibodies are typically monoclonal or polyclonal, targeting specific epitopes to ensure specificity. Their application enhances understanding of disease mechanisms and supports therapeutic development for sarcoglycanopathies.
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