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PHKG2 Rabbit pAb (bs-5010R)  
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產品編號 bs-5010R
英文名稱 PHKG2 Rabbit pAb
中文名稱 磷酸化酶激酶γ2
別    名 GSD9C; PHK gamma LT; PHK gamma T; phosphorylase b kinase gamma catalytic chain testis/liver isoform; phosphorylase b kinase gamma catalytic chain, liver/testis isoform; phosphorylase kinase gamma subunit 2; phosphorylase kinase subunit gamma 2; phosphorylase kinase, gamma 2(testis); PHKG2_HUMAN; phosphorylase kinase, gamma 2(testis/liver); PSK C3; Serine/threonine protein kinase PHKG2.  
研究領域 腫瘤  免疫學  信號轉導  激酶和磷酸酶  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human (predicted: Mouse,Rat,Rabbit,Pig,Cow,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 46 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PHKG2: 81-180/406 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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, and encoded by one gene. The gamma subunit also includes the skeletal muscle and hepatic isoforms, and the hepatic isoform is encoded by this gene. 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 9C, also known as autosomal liver glycogenosis. Alternatively spliced transcript variants encoding different isoforms have been identified in this gene.

Function:
Catalytic subunit of the phosphorylase b kinase (PHK), which mediates the neural and hormonal regulation of glycogen breakdown (glycogenolysis) by phosphorylating and thereby activating glycogen phosphorylase (PYGB, PYGL or PYGM). May regulate glycogeneolysis in the testis. In vitro, phosphorylates TNNI3, TNNT2, MAPT/TAU, GAP43 and NRGN/RC3.

Subunit:
Hexadecamer of 4 heterotetramers, each composed of alpha, beta, gamma, and delta subunits. Alpha (PHKA1 or PHKA2) and beta (PHKB) are regulatory subunits, gamma (PHKG1 or PHKG2) is the catalytic subunit, and delta is calmodulin.

DISEASE:
Defects in PHKG2 are a cause of glycogen storage disease type 9C (GSD9C) [MIM:613027]. A metabolic disorder manifesting in infancy with hepatomegaly, growth retardation, hypotonia, liver dysfunction, and elevated plasma aminotransferases and lipids. These symptoms improve with age in most cases; however, some patients may develop hepatic fibrosis or cirrhosis.

Similarity:
Belongs to the protein kinase superfamily. CAMK Ser/Thr protein kinase family.
Contains 1 protein kinase domain.

Gene ID:
5261

Database links:
UniProtKB/Swiss-Prot: P15735.1

產品圖片
Sample: 293T(Human) Cell Lysate at 30 ug Primary: Anti- PHKG2 (bs-5010R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 46 kD Observed band size: 44 kD
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