Anti-PHKA2 Antibody

The PHKA2 gene encodes the alpha-2 regulatory subunit of phosphorylase kinase, a serine/threonine kinase complex central to glycogen metabolism. Hormonal signals such as glucagon and epinephrine trigger phosphorylation of glycogen phosphorylase b by this complex, releasing glucose-1-phosphate for energy production. Mutations in this X-linked locus cause glycogen storage disease type IXa (GSD IXa, historically called XLG), with clinical features including childhood hepatomegaly, mild fasting hypoglycemia, elevated hepatic transaminases, growth retardation, and sometimes renal tubular dysfunction.

This Antibodies.com reagent is a rabbit polyclonal IgG raised against the human PHKA2 protein. It is supplied as 100 µl of unconjugated antiserum validated for Western blot detection of the approximately 130 kDa subunit in liver and skeletal muscle lysates. The polyclonal format provides recognition of multiple epitopes along the full-length protein, which can aid detection of splice isoforms and partially degraded fragments in patient samples.

Recommended applications include immunoblotting of patient-derived fibroblasts or hepatic biopsies to investigate suspected GSD IXa, characterization of phosphorylase kinase subunit composition in metabolic signaling studies, and basic research into glycogen mobilization. For storage, keep the vial at -20 °C in working aliquots to limit freeze-thaw cycles; short-term storage at 4 °C is acceptable for two to three weeks during active use. Research use only.