HPA009413
Anti-JPH1 antibody produced in rabbit
Ab1, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
别名:
Anti-JP-1 antibody produced in rabbit, Anti-Junctophilin type 1 antibody produced in rabbit, Anti-Junctophilin-1 antibody produced in rabbit
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所有图片(4)
About This Item
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生物源
rabbit
品質等級
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: 1:20-1:50
免疫原序列
PQSKYSGRHHIPNPSNGELHSQYHGYYVKLNAPQHPPVDVEDGDGSSQSSSALVHKPSANKWSPSKSVTKPVAKESKAEPKAKKSELAIPKNPASNDSCPAL
UniProt登錄號
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 ... JPH1(56704)
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一般說明
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)
應用
Anti-JPH1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . 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.
Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Western Blotting (1 paper)
Western Blotting (1 paper)
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 APREST71724
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
免責聲明
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.
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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)
Shinji Komazaki et al.
FEBS letters, 524(1-3), 225-229 (2002-07-24)
Junctophilins (JP-1, JP-2, and JP-3) are transmembrane proteins expressed in the junctional membrane complexes in excitable cells. Both JP-1 and JP-2 are co-expressed in the triads of skeletal muscle, but only JP-2 is expressed in cardiac muscle. We analyzed the
M Nishi et al.
Biochemical and biophysical research communications, 273(3), 920-927 (2000-07-13)
Junctophilin (JP) subtypes, namely JP-1, 2, and 3, have been currently identified in excitable cells and constitute a novel family of junctional membrane complex proteins. Our studies have suggested that JPs take part in the formation of junctional membrane complexes
Simona Boncompagni et al.
PloS one, 7(7), e39962-e39962 (2012-07-07)
Triadin (Tdn) and Junctin (Jct) are structurally related transmembrane proteins thought to be key mediators of structural and functional interactions between calsequestrin (CASQ) and ryanodine receptor (RyRs) at the junctional sarcoplasmic reticulum (jSR). However, the specific contribution of each protein
David Pla-Martín et al.
Human molecular genetics, 24(1), 213-229 (2014-08-30)
Mutations in the GDAP1 gene cause different forms of Charcot-Marie-Tooth (CMT) disease, and the primary clinical expression of this disease is markedly variable in the dominant inheritance form (CMT type 2K; CMT2K), in which carriers of the GDAP1 p.R120W mutation
Cecilia Lindskog et al.
BMC genomics, 16, 475-475 (2015-06-26)
To understand cardiac and skeletal muscle function, it is important to define and explore their molecular constituents and also to identify similarities and differences in the gene expression in these two different striated muscle tissues. Here, we have investigated the
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