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PHKB Rabbit anti-Human, Mouse, Polyclonal, Proteintech

Product Code. 16841594 Shop All Proteintech Products
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16841594 20 μL 20µL
16831594 150 μL 150µL
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Product Code. 16841594 Supplier Proteintech Supplier No. 134001AP20UL

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Rabbit Polyclonal Antibody

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.

Specifications

Antigen PHKB
Applications Immunoprecipitation, Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 0.24 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene PHKB
Gene Accession No. Q7TSH2, Q93100
Gene Alias PHKB, phosphorylase kinase, beta
Gene Symbols PHKB
Host Species Rabbit
Immunogen PHKB Fusion Protein Ag4240
Purification Method Antigen Affinity Chromatography
Quantity 20 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 102093, 5257
Target Species Human, Mouse
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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