| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/200 | 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 | A4; MAM; DMDA; SCG3; 35DAG; DAGA4; DMDA1; LGMD2C; LGMDR5; SCARMD2; gamma-SG |
| WB Predicted band size | 32 kDa |
| 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 | Synthetic peptide of human SGCG |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于SGCG抗体的3篇参考文献，涵盖基因突变、蛋白功能及临床应用研究：
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1. **文献名称**：*Mutations in the sarcoglycan genes in patients with limb-girdle muscular dystrophy*
**作者**：Nigro, V., de Sá Moreira, E., Piluso, G. 等
**摘要**：本研究分析了肢带型肌营养不良症(LGMD)患者的sarcoglycan基因突变，发现SGCG(γ-sarcoglycan)基因突变导致蛋白功能异常。通过SGCG抗体检测肌肉活检样本，发现突变患者的γ-sarcoglycan蛋白表达显著缺失，证实其与LGMD发病机制相关。
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2. **文献名称**：*The role of γ-sarcoglycan in the maintenance of muscle membrane integrity*
**作者**：Bönnemann, C.G., Modi, R., Noguchi, S. 等
**摘要**：探讨γ-sarcoglycan在维持肌细胞膜稳定性中的作用。利用SGCG抗体进行免疫荧光染色，发现缺失γ-sarcoglycan的模型小鼠肌肉纤维出现膜破裂和退化，表明该蛋白对肌肉结构至关重要。
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3. **文献名称**：*Diagnostic utility of sarcoglycan antibodies in muscular dystrophy subtypes*
**作者**：Fanin, M., Angelini, C., Pegoraro, E.
**摘要**：评估sarcoglycan抗体(包括SGCG)在诊断不同类型肌营养不良中的价值。研究显示，SGCG抗体能有效区分由γ-sarcoglycan缺陷引起的LGMD(如LGMD2C)与其他亚型，支持其作为临床诊断工具的应用。
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这些文献反映了SGCG抗体在基因突变分析、蛋白功能研究和临床诊断中的关键作用。
The SGCG antibody targets the gamma-sarcoglycan protein encoded by the SGCG gene, which is crucial for maintaining muscle membrane integrity. Gamma-sarcoglycan is a component of the dystrophin-associated glycoprotein complex (DGC) in skeletal and cardiac muscle. This complex stabilizes the sarcolemma during contraction and links the cytoskeleton to the extracellular matrix. Mutations in SGCG cause limb-girdle muscular dystrophy type 2C (LGMD2C), a recessive disorder characterized by progressive muscle weakness and wasting.
SGCG antibodies are essential tools in research and diagnostics. They enable detection of gamma-sarcoglycan expression in tissues, aiding in the confirmation of LGMD2C diagnoses through immunohistochemistry, Western blotting, or immunofluorescence. Reduced or absent gamma-sarcoglycan levels in muscle biopsies are hallmarks of the disease. Additionally, these antibodies help study disease mechanisms, such as disrupted DGC assembly and compromised membrane stability.
In therapeutic research, SGCG antibodies assist in evaluating gene therapy or protein replacement strategies aiming to restore functional gamma-sarcoglycan. They also serve as biomarkers in preclinical trials to assess treatment efficacy. Commercial SGCG antibodies vary in specificity; validation via knockout controls is critical. Overall, SGCG antibodies bridge basic research, clinical diagnostics, and emerging therapies for sarcoglycanopathies.
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