| 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 | PHK; PYK; XLG; PYKL; XLG2; GSD9A |
| 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 |
| Immunogen | Synthetic peptide of human PHKA2 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于PHKA2抗体的3篇示例文献(内容为虚构，供参考)：
1. **文献名称**：*PHKA2 mutations and antibody-based detection in glycogen storage disease type IX*
**作者**：Smith A, et al. (2018)
**摘要**：本研究开发了一种特异性PHKA2抗体，用于检测肝组织中PHKA2蛋白表达水平。通过免疫组化分析，发现IX型糖原贮积症患者的PHKA2表达显著降低，为疾病诊断提供新工具。
2. **文献名称**：*Development of a monoclonal antibody against human PHKA2 for functional studies*
**作者**：Chen L, et al. (2020)
**摘要**：报道了一种高特异性PHKA2单克隆抗体的制备，并通过Western blot和免疫荧光验证其可靠性。该抗体成功用于研究PHKA2在糖原代谢调控中的分子机制。
3. **文献名称**：*PHKA2 antibody reveals tissue-specific phosphorylation patterns in metabolic disorders*
**作者**：Wang Y, et al. (2022)
**摘要**：利用PHKA2抗体分析不同组织中PHKA2磷酸化状态，发现其在肝脏和肌肉中的修饰差异与糖原分解异常相关，为代谢疾病的靶向治疗提供依据。
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注：以上文献为示例，实际研究中建议通过PubMed或Web of Science检索真实文献(关键词：PHKA2 antibody, glycogen storage disease)。如需具体文献协助，可进一步说明需求。
The PHKA2 antibody targets the protein encoded by the PHKA2 gene, which is a critical subunit of phosphorylase kinase (PhK). Phosphorylase kinase is a hexadecameric enzyme complex (αβγδ)₄ that regulates glycogen metabolism by activating glycogen phosphorylase, a rate-limiting enzyme in glycogen breakdown. The PHKA2 gene encodes the α-subunit, specifically the α2 isoform predominantly expressed in the liver. This subunit plays a regulatory role, modulating PhK activity through calcium binding and interaction with other subunits.
Mutations in PHKA2 are linked to glycogen storage disease type IXa (GSD IXa), an X-linked recessive disorder characterized by hepatomegaly, growth retardation, and elevated liver enzymes due to impaired glycogenolysis. PHKA2 antibodies are essential tools in research and diagnostics, enabling the detection of PHKA2 protein expression levels, localization, and potential mutations. They are used in techniques like Western blotting, immunohistochemistry, and ELISA to study PhK complex integrity, tissue-specific expression, and disease mechanisms.
Studies utilizing PHKA2 antibodies have advanced understanding of liver-specific glycogen metabolism dysregulation and contributed to the development of targeted therapies for GSDs. These antibodies also aid in distinguishing PHKA2-related disorders from other metabolic conditions with overlapping clinical features.
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