Anti-PHKB Rabbit Polyclonal Antibody

Supplier: Proteintech
10092-200EA 402 USD
Anti-PHKB Rabbit Polyclonal Antibody
PHKB gene encodes phosphorylase kinase subunit beta involved in glycan biosynthesis and glycogen metabolism. PHKB activity is regulated by phosphorylation of various serine residues, and catalyzes the phosphorylation of serine in certain substrates, including troponin I. Phosphorylase kinase (PhK) complex, composed of alpha, beta, gamma, and delta subunits, stimulates energy production from glycogen in the cascade activation of glycogenolysis. Its large homologous alpha and beta subunits regulate the activity of the catalytic gamma subunit. Defects in PHKB are the cause of glycogen storage disease type 9B (GSD9B) also known as phosphorylase kinase deficiency of liver and muscle (PKD), characterized by hepathomegaly, only slightly elevated transaminases and plasma lipids, clinical improvement with increasing age, and remarkably no clinical muscle involvement.

Western Blot: Mouse Heart Tissue, 1:200-1:2000; IHC: Human Skeletal Muscle Tissue, 1:20-1:200

Type: Primary
Antigen: PHKB
Clonality: Polyclonal
Conjugation: Unconjugated
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat
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