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PHKB pAb

PHKB pAb

Cat no: BS5854

Supplier: Bioworld Technology, Inc.
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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.
Catalogue number: BS5854
Reactivities: Human, Mouse, Rat
Hosts: Rabbit
Applications: Western Blot
Size: 100ug/100ul
Swiss prot: Q93100
Dilutions: WB: 1:500~1:1000
Form: liquid
Storage buffer: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Concentration: 1ug/ul
Antigen: Synthetic peptide, corresponding to amino acids 699-744 of Human PHKB.
Species: PHKB pAb detects endogenous levels of PHKB protein.
Molecular weight: ~ 125 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage temp: Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.
Alternative names: Phosphorylase b kinase regulatory subunit beta; Phosphorylase kinase subunit beta; Phosphorylase B
Additional info: For research use only, not for use in diagnostic procedure.

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