Thermo Fisher Scientific PHKB Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:1,000

-

Product Specifications

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

A synthetic peptide derived from the internal region of human KPBB

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Storage conditions

-20°C

Shipping conditions

Wet ice

RRID

AB_2555351

Target Information

Phosphorylase kinase is a hexadecameric enzyme that is comprised of four copies of four subunits that are encoded by four separate genes: PHKA, PHKB, PHKG, and PHKD. This serine/threonine specific kinase converts glycogen phosphorylase b to glycogen phosphorylase a, resulting in the release of glucose-1-phophate from glycogen. PHKB (Phosphorylase b kinase regulatory subunit beta) is a 1093 amino acid subunit of phosphorylase kinase that, along with PHKA, has regulatory functions controlled by phosphorylation. Defects in the gene encoding PHKB are the cause of glycogen storage disease type 9B, which is also known as phosphorylase kinase deficiency of liver and muscle. This disease is characterized by a mild phenotype of hepatomegaly with only slightly elevated transaminase and plasma lipids, no clinical muscle involvement, and generally is correlated with a gradual improvement with increasing age. There are four isoforms of PHKB that are produced as a result of alternative splicing events.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.