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货号： bs-5010R-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-5010R-Cy7
- 英文名称
- Anti-PHKG2/Cy7
- 中文名称
- Cy7标记的磷酸化酶激酶γ2
- 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 免疫学 信号转导 激酶和磷酸酶 新陈代谢
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
- 产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 46kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 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 TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 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.
- 产品介绍
- background:
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.
Database links:
UniProtKB/Swiss-Prot: P15735.1
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.