Synthetic peptide directed towards the N terminal region of human JPH3
Application
Anti-JPH3 antibody produced in rabbit is suitable for western blotting at a concentration of 1.0μg/ml.
Biochem/physiol Actions
Junctophilins are localized to the endoplasmic/sarcoplasmic reticulum membrane and anchor it to the plasma membrane. Mutations in Junctophilin 3 (JPH3) induces polyglutamine protein toxicity in Huntington′s disease.
Sequence
Synthetic peptide located within the following region: SSGGRFNFDDGGSYCGGWEDGKAHGHGVCTGPKGQGEYTGSWSHGFEVLG
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Disclaimer
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.
Huntington's disease-like-2 (HDL2) is a phenocopy of Huntington's disease caused by CTG/CAG repeat expansion at the Junctophilin-3 (JPH3) locus. The mechanisms underlying HDL2 pathogenesis remain unclear. Here we developed a BAC transgenic mouse model of HDL2 (BAC-HDL2) that exhibits progressive
The Journal of biological chemistry, 286(51), 43717-43725 (2011-10-25)
Junctophilins (JPs) anchor the endo/sarcoplasmic reticulum to the plasma membrane, thus contributing to the assembly of junctional membrane complexes in striated muscles and neurons. Recent studies have shown that JPs may be also involved in regulating Ca2+ homeostasis. Here, we
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