PHKB rabbit pAb

PHKB rabbit pAb

AO-06-ES6585-100

PHKB rabbit pAb 100μL

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Antibody Product Overview

ELK.NoES6585
Product namePHKB rabbit pAb
ReactivityHuman;Mouse
ApplicationsWB;IHC
Other namePHKB; Phosphorylase b kinase regulatory subunit beta; Phosphorylase kinase subunit beta
Size100μL
Unit price ($)248
Human gene ID5257
Human Swiss-ProtQ93100
SourceRabbit
IsotypeIgG
TargetPHKB
Fields>>Calcium signaling pathway;>>Insulin signaling pathway;>>Glucagon signaling pathway
Gene namePHKB
Protein namePhosphorylase b kinase regulatory subunit beta
Human gene linkView Human Gene
Human Swiss linkView Human Swiss-Prot
Mouse gene ID102093
Mouse gene linkView Mouse Gene
Mouse Swiss-ProtQ7TSH2
Mouse Swiss linkView Mouse Swiss-Prot
Rat gene ID
Rat gene link
Rat Swiss-Prot
Rat Swiss link
ImmunogenThe antiserum was produced against synthesized peptide derived from human KPBB. AA range:661-710
SpecificityPHKB Polyclonal Antibody detects endogenous levels of PHKB protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
ClonalityPolyclonal
DilutionWB 1:500-2000;IHC-p 1:50-300
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration1 mg/ml
Storage stability-20°C/1 year
Molecular Weight (Da)
Observed band (KD)124kD
BackgroundPhosphorylase 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 deficiency
Functiondisease: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.,similarit
Subcellular locationCell membrane ; Lipid-anchor ; Cytoplasmic side .
ExpressionUterus,

Additional Images

Image 1
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Western blot analysis of lysates from K562 cells, using KPBB Antibody. The lane on the right is blocked with the synthesized peptide.
Image 2
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Immunohistochemical analysis of paraffin-embedded human Squamous cell carcinoma of lung. 1, Antibody was diluted at 1:200(4° overnight). 2, Tris-EDTA,pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 45min).
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: AO-06-ES6585-100
: 10 Produits
Hurry! only 10 items left in stock.

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