Vergleich

PHKG2 (phosphorylase kinase. gamma 2 (testis))

ArtNr 18-003-42332
Hersteller GENWAY
Menge 0.05 mg
Kategorie
Typ Antibody
Applikationen WB, IHC
Specific against other
ECLASS 10.1 32160702
ECLASS 11.0 32160702
UNSPSC 12352203
Alias GWB-AE6E22
Similar products 18-003-42332
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Genway ID:
GWB-AE6E22
Antigen Specificity:
Polyclonal antibody produced in rabbits immunized with a synthetic peptide corresponding to a region of Human PHKG2. Western Blot: Suggested dilution at 0. 5 microg/ml in 5% skim milk / PBS buffer. and HRP conjugated anti-Rabbit IgG should be diluted in 1: 50. 000 - 100. 000 as second antibody.
ELISA Titre:
1:62500
Category:
Protein Kinases
Note:
Suggested starting concentrations are provided. Optimal dilutions should be determined by end-user. Differences in calculated versus apparent molecular weight may be due to post-translational modifications or protein hydrophobicity. Mutations in PHKG2 along with PHKA2 and PHKB. all three different genes of phosphorylase kinase (Phk) subunits. can give rise to glycogen storage disease of the liver. The autosomal-recessive. liver-specific variant of Phk deficiency is caused by mutation
Function:
Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates including troponin I. This isozyme may regulate glycogeneolysis in the testis.
Catalytic Activity:
2 ATP + phosphorylase b = 2 ADP + phosphorylase a.
Subunit:
Polymer of 16 chains four each of alpha beta gamma and delta. Alpha and beta are regulatory chains gamma is the catalytic chain and delta is calmodulin.
Disease:
Defects in PHKG2 are a cause of glycogen storage disease type 9C (GSD9C) [MIM:172471]; also known as autosomal liver glycogenosis. GSD9C is a metabolic disorder manifesting in infancy with hepatomegaly growth retardation and elevated plasma aminotransferases and lipids.
Similarity:
Belongs to the protein kinase superfamily. CAMK Ser/Thr protein kinase family.
Similarity:
Contains 1 protein kinase domain. Burwinkel. B. . et al. . (1998) Hum. Mol. Genet. 7 (1). 149-154.

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Menge: 0.05 mg
Lieferbar: In stock
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