磷酸化酶激酶γ2说明书 英文名称  Anti-PHKG2

中文名称  磷酸化酶激酶γ2说明书 

别    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.

产品属性：

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit

产品类型   一抗  

研究领域     免疫学 信号转导 激酶和磷酸酶

蛋白分子量  predicted molecular weight: 46kDa 

性    状   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human PHKG2

亚    型  IgG

纯化方法   affinity purified by Protein A

储 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

磷酸化酶激酶γ2说明书 产品应用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品介绍 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.

Contains 1 protein kinase domain.

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

实验流程：

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