| WB | 1/1000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 1/10-1/50 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | 4-alpha-glucan-branching enzyme, Brancher enzyme, Glycogen-branching enzyme, GBE1 |
| Entrez GeneID | 2632 |
| WB Predicted band size | 80.5kDa |
| 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 | This GBE1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 527-556 amino acids from the Central region of human GBE1. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于GBE1抗体的3篇参考文献的简要列举(示例格式，非真实文献)：
1. **文献名称**：*GBE1 antibody validation in glycogen storage disease type IV models*
**作者**：Smith A, et al.
**摘要**：研究通过免疫印迹和免疫组化验证了GBE1抗体的特异性，发现其在患者肝组织样本中能有效检测糖原分支酶的表达缺失，支持其在诊断中的应用。
2. **文献名称**：*Structural insights into GBE1 function using monoclonal antibodies*
**作者**：Chen L, et al.
**摘要**：通过开发高特异性单克隆抗体，揭示了GBE1蛋白的活性结构域，并证明抗体可抑制异常糖原聚集，为治疗干预提供新靶点。
3. **文献名称**：*GBE1 deficiency and cardiomyopathy: Antibody-based mechanistic study*
**作者**：Rodriguez M, et al.
**摘要**：利用GBE1抗体分析基因敲除小鼠模型，发现酶活性降低导致心肌糖原沉积异常，揭示了IV型糖原累积病中心脏病变的分子机制。
(注：以上为示例内容，实际文献需通过PubMed或学术数据库检索确认。)
GBE1 (Glycogen Branching Enzyme 1) is a critical enzyme encoded by the *GBE1* gene, responsible for introducing branch points in glycogen molecules during synthesis. This branching is essential for glycogen solubility and efficient energy storage/release. Mutations in *GBE1* are linked to glycogen storage disease type IV (GSD IV, Andersen disease), a rare autosomal recessive disorder characterized by progressive liver dysfunction, neuromuscular impairments, and cardiomyopathy due to abnormal glycogen accumulation.
GBE1 antibodies are immunological tools designed to detect and quantify GBE1 protein expression in research and diagnostic contexts. They are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to study tissue-specific GBE1 distribution, expression levels under metabolic stress, or disease states. In research, these antibodies help elucidate GSD IV pathophysiology, including how *GBE1* mutations disrupt glycogen structure and cellular metabolism. Additionally, GBE1 antibodies are employed in cancer studies, as altered glycogen metabolism is observed in tumors like hepatocellular carcinoma and breast cancer, where GBE1 dysregulation may influence malignancy or therapeutic resistance.
Recent studies also explore GBE1's role beyond glycogen metabolism, such as its interaction with autophagy pathways or response to cellular energy demands. High-specificity GBE1 antibodies are crucial for distinguishing functional isoforms or post-translational modifications, aiding in the development of targeted therapies for metabolic disorders and cancers.
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