| 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 | 1/1000-1/2000 | Human,Mouse,Rat |
| Aliases | PHKA |
| WB Predicted band size | 137 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, Rat |
| Immunogen | Synthetic peptide of human PHKA1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
+ +
以下是关于PHKA1抗体的模拟参考文献示例(注：内容为虚构，仅作格式参考)：
---
1. **文献名称**: "Characterization of PHKA1-Specific Antibodies for Detection of Phosphorylase Kinase in Skeletal Muscle"
**作者**: Smith J, et al.
**摘要**: 本研究开发并验证了一种高特异性的PHKA1抗体，用于Western blot和免疫组化分析。研究发现PHKA1在骨骼肌中高表达，其表达水平与糖原代谢活性相关，为研究糖原贮积症IX型提供了工具。
2. **文献名称**: "PHKA1 Mutations and Protein Expression Analysis Using Monoclonal Antibodies in Glycogen Storage Disease"
**作者**: Lee H, et al.
**摘要**: 通过PHKA1特异性抗体检测患者肌肉样本，发现PHKA1蛋白表达缺失与基因突变相关。该抗体在糖原贮积症IX型的分子诊断和病理机制研究中具有重要应用价值。
3. **文献名称**: "Subcellular Localization of PHKA1 in Hepatocytes Revealed by Immunofluorescence Staining"
**作者**: Garcia R, et al.
**摘要**: 利用PHKA1抗体进行免疫荧光实验，揭示了PHKA1在肝细胞中的亚细胞定位，表明其与糖原颗粒的调控密切相关，为肝脏糖原代谢研究提供了新见解。
4. **文献名称**: "Development of a PHKA1 Knockout Mouse Model: Antibody-Based Validation of Gene Silencing"
**作者**: Tanaka M, et al.
**摘要**: 通过PHKA1抗体验证了基因敲除小鼠模型中蛋白表达的缺失，证实PHKA1缺失导致磷酸化酶激酶活性下降，影响糖原分解代谢通路。
---
如需真实文献，建议在 **PubMed** 或 **Google Scholar** 中搜索关键词“PHKA1 antibody”、“phosphorylase kinase alpha 1” + “antibody”，筛选涉及抗体应用、疾病机制或蛋白功能的研究。
The PHKA1 antibody targets the phosphorylase kinase regulatory subunit alpha 1 (PHKA1), a critical component of the phosphorylase kinase (PhK) complex. This tetrameric enzyme, composed of α, β, γ, and δ subunits, regulates glycogen metabolism by activating glycogen phosphorylase, which initiates glycogen breakdown. The α subunit, encoded by PHKA1. plays a regulatory role, modulating PhK activity in response to calcium and ATP levels. PHKA1 is predominantly expressed in skeletal muscle, where it ensures energy homeostasis during muscle contraction.
Mutations in PHKA1 are linked to glycogen storage disease type IXd (GSD IXd), a rare inherited disorder characterized by exercise intolerance, muscle weakness, and elevated glycogen accumulation. Research on PHKA1 antibodies focuses on understanding its expression, post-translational modifications, and interactions within the PhK complex. These antibodies are essential tools in Western blotting, immunohistochemistry, and immunofluorescence to study tissue-specific expression patterns or pathological changes in muscle biopsies from GSD IXd patients.
PHKA1 antibodies also aid in elucidating the molecular mechanisms underlying PhK dysfunction and its role in metabolic regulation. Their application extends to preclinical studies aiming to develop therapeutic strategies for glycogen-related disorders. However, challenges remain in ensuring antibody specificity due to structural similarities among PhK subunits. Validated PHKA1 antibodies are crucial for advancing research into muscle metabolism and genetic metabolic diseases.
×
