产品说明
一般描述
The gene PHKA1 (phosphorylase kinase, α 1) encodes the skeletal muscle isoform of the α subunit of phosphorylase kinase. This enzyme is a hexadekameric protein with 16 subunits containing four each of α, β, γ and δ. The gene is mapped to chromosome X in humans.
免疫原
Phosphorylase b kinase regulatory subunit-α, skeletal muscle isoform recombinant protein epitope signature tag (PrEST)
应用
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org)and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
生化/生理作用
The hexadekameric complex of phosphorylase kinase (Phk) is involved in the regulation of glycogen breakdown by phosphorylation of glycogen phosphorylase. Mutation in the PHKA1 (phosphorylase kinase, α 1) gene can cause a deficiency of Phk leading to glycogen storage diseases or muscle-specific glycogenosis. This is characterized by exercise intolerance with early fatigue, pain, cramps and rarely myoglobinuria.
特点和优势
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
联系
Corresponding Antigen APREST74010.
外形
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
法律信息
Prestige Antibodies is a registered trademark of sigma-aldrich Co. LLC
基本信息
人类蛋白质图谱编号 | HPA001081 Human Protein Atlas characterization data |
产品性质
生物来源 | rabbit |
质量水平 | 100 |
偶联物 | unconjugated |
抗体形式 | affinity isolated antibody |
antibody product type | primary antibodies |
克隆 | polyclonal |
产品线 | Prestige Antibodies® Powered by Atlas Antibodies |
形式 | buffered aqueous glycerol solution |
species reactivity | human |
包装 | antibody small pack of 25 μL |
增强验证 | orthogonal RNAseq Learn more about Antibody Enhanced Validation |
technique(s) | immunofluorescence: 0.25-2 μg/mL immunohistochemistry: 1:50-1:200 western blot: 0.04-0.4 μg/mL |
免疫原序列 | AHSLRCSAEEATEGLMNLSPSAMKNLLHHILSGKEFGVERSVRPTDSNVSPAISIHEIGAVGATKTERTGIMQLKSEIKQSPGTSMTPSSGSFPSAYDQQSSKDSRQGQWQ |
运输 | wet ice |
储存温度 | −20℃ |
Gene Information | human ... PHKA1P1(646780) |
安全信息
储存分类代码 | 10 - Combustible liquids |
WGK | WGK 1 |
闪点(F) | Not applicable |
闪点(C) | Not applicable |
个人防护装备 | Eyeshields, Gloves, multi-purpose combination respirator cartridge (US) |
Sigma-Aldrich