产品说明
一般描述
JPH1 (junctophilin 1) is a subtype of the junctional membrane complex protein called JP, which contains three subtypes. This subtype is predominantly expressed in skeletal muscles. It is expressed in the junctional membrane complexes of excitable cells, and exists as a transmembrane protein. This gene is localized to human chromosome 8q21.1.
免疫原
Junctophilin-1 recombinant protein epitope signature tag (PrEST)
应用
Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Western Blotting (1 paper)
Anti-JPH1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org). Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
生化/生理作用
Junctophilins span the membrane of intracellular Ca2+ storage vesicles and aids in their interaction with cell-surface membrane, thus, facilitating the formation of junctional membrane complexes. Studies in mice show that this protein plays a crucial role in the formation of triads during skeletal muscle development. JPH1 and GADP-1 are parts of a common pathway, and hence, interdependent. Thus, mutations in GDAP-1 (ganglioside-induced differentiation-associated protein 1) might phenotypically be manifested by JPH1. Therefore, JPH1 is implicated in Charcot-Marie-Tooth (CMT) disease.
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 APREST71724.
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
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
人类蛋白质图谱编号 | HPA009413 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: 1:20-1:50 |
免疫原序列 | PQSKYSGRHHIPNPSNGELHSQYHGYYVKLNAPQHPPVDVEDGDGSSQSSSALVHKPSANKWSPSKSVTKPVAKESKAEPKAKKSELAIPKNPASNDSCPAL |
UniProt登记号 | Q9HDC5 |
运输 | wet ice |
储存温度 | −20℃ |
Gene Information | human ... JPH1(56704) |
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