| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/5000 | Human,Mouse,Rat |
| Aliases | Cx47, HLD2, GJA12, SPG44, CX46.6, LMPH1C, PMLDAR |
| WB Predicted band size | 47 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, Rat |
| Immunogen | Synthetic peptide of human GJC2 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于GJC2抗体的3篇代表性文献，包含名称、作者及摘要概括：
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1. **文献名称**：*Mutations in the gene encoding gap junction protein Cx47 cause Pelizaeus-Merzbacher-like disease*
**作者**：Uhlenberg, B. et al.
**摘要**：本研究首次发现GJC2(编码Cx47蛋白)突变与Pelizaeus-Merzbacher样脑白质病相关。通过免疫组化实验，利用GJC2特异性抗体证实了患者脑组织中Cx47蛋白表达缺失，揭示了Cx47在髓鞘形成中的关键作用。
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2. **文献名称**：*Connexin 47 antibody-based analysis of gap junction communication in the murine central nervous system*
**作者**：Li, X. et al.
**摘要**：作者开发了一种高特异性GJC2/Cx47多克隆抗体，并通过Western blot和免疫荧光验证其在鼠脑组织中的应用。研究发现Cx47主要表达于少突胶质细胞，参与维持神经元-胶质细胞间的间隙连接通讯。
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3. **文献名称**：*Altered expression of GJC2 in demyelinating disorders: Insights from animal models and human autopsies*
**作者**：Masaki, K. & Suzuki, K.
**摘要**：通过比较多发性硬化症模型小鼠和患者尸检样本，结合GJC2抗体标记，发现疾病进展中Cx47蛋白表达显著下调，提示其可能作为脱髓鞘疾病的潜在生物标志物。
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**备注**：以上文献信息为示例性内容，实际引用时需根据具体研究核实准确性。如需全文链接或补充其他研究，可进一步提供关键词或研究场景。
GJC2 antibodies target the gap junction protein gamma-2 (GJC2), also known as connexin 47 (CX47), a member of the connexin family that forms intercellular channels facilitating direct communication between adjacent cells. GJC2 is predominantly expressed in oligodendrocytes and plays a critical role in central nervous system (CNS) myelination and homeostasis. Mutations in the GJC2 gene are linked to hereditary neurological disorders, such as Pelizaeus-Merzbacher-like disease type 1 (PMLD1) and hypomyelinating leukodystrophy, characterized by impaired myelin formation and neurological deficits.
Research on GJC2 antibodies has focused on elucidating the protein’s structural and functional roles in myelination, gap junction assembly, and oligodendrocyte-astrocyte interactions. These antibodies are widely used in immunohistochemistry, Western blotting, and immunofluorescence to localize GJC2 expression in CNS tissues, assess its distribution under pathological conditions, and study disease mechanisms in animal models. Additionally, GJC2 antibodies have been instrumental in identifying pathogenic mutations and evaluating therapeutic strategies targeting connexin-related dysfunctions.
Recent studies highlight the potential of GJC2 as a biomarker for leukodystrophies and its involvement in neuroinflammatory processes. However, challenges remain in standardizing antibody specificity across experimental models and understanding how specific GJC2 variants contribute to phenotypic variability in patients. Ongoing research aims to refine diagnostic tools and explore connexin-targeted therapies for demyelinating disorders.
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