HPA001081
Anti-PHKA1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
别名:
Anti-Phosphorylase b kinase regulatory subunit-α, skeletal muscle isoform antibody produced in rabbit, Anti-Phosphorylase kinase-α M subunit antibody produced in rabbit
登录查看公司和协议定价
所有图片(5)
About This Item
生物源
rabbit
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
產品線
Prestige Antibodies® Powered by Atlas Antibodies
形狀
buffered aqueous glycerol solution
物種活性
human
加強驗證
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
技術
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
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... PHKA1P1(646780)
相关类别
一般說明
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)
應用
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project 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. We also provide Prestige Antibodies® protocols and other useful information.
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. We also provide Prestige Antibodies® protocols and other useful information.
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.
特點和優勢
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:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
聯結
Corresponding Antigen APREST74010
外觀
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
法律資訊
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
未找到合适的产品？
试试我们的产品选型工具.
儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 1
閃點（°F）
Not applicable
閃點（°C）
Not applicable
個人防護裝備
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
J B Lyon et al.
Science (New York, N.Y.), 155(3769), 1550-1551 (1967-03-24)
The gene for phosphoryl ase b kinase, a skeletal muscle enzyme, has been located on the X chromo some of mice. The inheritance of the enzyme through two generations from original matings between one inbred strain of mice, the I
A Schneider et al.
Nature genetics, 5(4), 381-385 (1993-12-01)
Heritable phosphorylase kinase (Phk) deficiency underlies a group of glycogenoses in humans, mice and rats that differ in mode of inheritance and tissue-specificity. It is assumed that this heterogeneity is caused by mutations affecting different subunits and isoforms of Phk.
Barbara Burwinkel et al.
European journal of human genetics : EJHG, 11(7), 516-526 (2003-06-26)
Muscle-specific deficiency of phosphorylase kinase (Phk) causes glycogen storage disease, clinically manifesting in exercise intolerance with early fatiguability, pain, cramps and occasionally myoglobinuria. In two patients and in a mouse mutant with muscle Phk deficiency, mutations were previously found in
我们的科学家团队拥有各种研究领域经验，包括生命科学、材料科学、化学合成、色谱、分析及许多其他领域.
联系技术服务部门