PHKA2抗体

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     The image is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using P13378(F13B Antibody) at dilution 1/20. (Original magnification: ×200)    

  
  

PHKA2 (phosphorylase kinase subunit alpha 2) is a critical component of the phosphorylase kinase (PhK) enzyme complex, which regulates glycogen metabolism by activating glycogen phosphorylase. This enzyme plays a key role in the breakdown of glycogen to glucose-1-phosphate, a process vital for maintaining energy homeostasis, particularly in liver and muscle tissues. The PHKA2 gene, located on the X chromosome (Xp22.2-p22.1), encodes the alpha subunit of PhK, serving as a regulatory element that modulates the enzyme’s activity through calcium and phosphorylation-dependent mechanisms.

Mutations in PHKA2 are linked to X-linked liver glycogen storage disease type IXa (GSD IXa), a rare inherited disorder characterized by hepatomegaly, growth retardation, and fasting hypoglycemia due to impaired glycogenolysis. PHKA2 antibodies are essential tools in research and diagnostics, enabling the detection of PHKA2 protein expression, localization, and functional studies. These antibodies aid in elucidating molecular mechanisms underlying GSD IXa, assessing mutation impacts on protein stability or activity, and developing targeted therapies. Commercial PHKA2 antibodies are typically developed using immunogenic peptides or recombinant proteins, validated for applications like Western blotting, immunohistochemistry, and immunofluorescence. Understanding PHKA2’s role through antibody-based studies contributes to advancing metabolic disease research and precision medicine approaches.