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PHKB polyclonal antibody

CAT: 0384-BS5854-01Size: 50 µLDry Ice: NoHazardous: No
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CAT#:0384-BS5854-01Size:50 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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Background
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.
CAS Number
9007-83-4
Structure Composition
Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Product Name Alternative
Phosphorylase b kinase regulatory subunit beta; Phosphorylase kinase subunit beta; Phosphorylase B
Swiss Prot
Q93100
Reactivity
Human, Mouse, Rat
Immunogen
Synthetic peptide, corresponding to amino acids 699-744 of Human PHKB.
Conjugation
Unconjugated
Applications
WB
Dilution
WB: 1:500~1:1000
Purity
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Modification
Unmodification
Molecular Weight
~ 125 kDa
Storage Conditions
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Notes
For research use only, not for use in diagnostic procedure.
Specificity
PHKB polyclonal antibody detects endogenous levels of PHKB protein.
Applications Notes
Western blot (WB) analysis of PHKB polyclonal antibody at 1:500 dilution Lane1:Hela cell lysate Lane2:sp2/0 cell lysate Lane3:H9C2 cell lysate
Host or Source
Rabbit

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