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PHKB Polyclonal Antibody
Datasheet
Catalog Number:
E20-73701Amount:
100ulPrice:
225$Purification:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.Immunogen:
The antiserum was produced against synthesized peptide derived from human KPBB. AA range:661-710Source:
Polyclonal, RabbitGene Name:
PHKBDilution:
Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.
- Western blot analysis of lysates from K562 cells, using KPBB Antibody. The lane on the right is blocked with the synthesized peptide.
Background:
phosphorylase kinase regulatory subunit beta(PHKB) Homo sapiens Phosphorylase kinase is a polymer of 16 subunits, four each of alpha, beta, gamma and delta. The alpha subunit includes the skeletal muscle and hepatic isoforms, encoded by two different genes. The beta subunit is the same in both the muscle and hepatic isoforms, encoded by this gene, which is a member of the phosphorylase b kinase regulatory subunit family. The gamma subunit also includes the skeletal muscle and hepatic isoforms, encoded by two different genes. The delta subunit is a calmodulin and can be encoded by three different genes. The gamma subunits contain the active site of the enzyme, whereas the alpha and beta subunits have regulatory functions controlled by phosphorylation. The delta subunit mediates the dependence of the enzyme on calcium concentration. Mutations in this gene cause glycogen storage disease type 9B, also known as phosphorylase kinase deficiencyProtein Name:
Phosphorylase b kinase regulatory subunit betaHuman Gene Id:
5257Human Swiss Prot No:
Q93100Mouse Gene Id:
102093Mouse Swiss Prot No:
Q7TSH2Specificity:
PHKB Polyclonal Antibody detects endogenous levels of PHKB protein.Formulation:
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.Concentration:
1 mg/mlStorage Stability:
-20°C/1 yearOther Name:
PHKB; Phosphorylase b kinase regulatory subunit beta; Phosphorylase kinase subunit betaMolecularWeight(Da):
124884Observed Band(KD):
124Function:
disease:Defects in PHKB are the cause of glycogen storage disease type 9B (GSD9B) [MIM:261750]; also known as phosphorylase kinase deficiency of liver and muscle (PKD). GSD9B is a metabolic disorder characterized by hepathomegaly, only slightly elevated transaminases and plasma lipids, clinical improvement with increasing age, and remarkably no clinical muscle involvement. Biochemical observations suggest that this mild phenotype is caused by an incomplete holoenzyme that lacks the beta subunit, but that may possess residual activity.,enzyme regulation:By phosphorylation of various serine residues.,function:Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The beta chain acts as a regulatory unit and modulates the activity of the holoenzyme in response to phosphorylation.,pathway:Glycan biosynthesis; glycogen metabolism.,similaritSubcellular Location:
cytosol,plasma membrane,phosphorylase kinase complex,Expression:
Uterus,
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Catalog Number
- Amount:100ul
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