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Junctional complexes between the plasma membrane and endoplasmic/sarcoplasmic reticulum are a common feature of all excitable cell types and mediate cross talk between cell surface and intracellular ion channels. Additionally we are shipping Junctophilin 2 Proteins (6) and many more products for this protein.
Showing 10 out of 86 products:
Cow (Bovine) Polyclonal JPH2 Primary Antibody for WB - ABIN2784534
Landstrom, Weisleder, Batalden, Bos, Tester, Ommen, Wehrens, Claycomb, Ko, Hwang, Pan, Ma, Ackerman: Mutations in JPH2-encoded junctophilin-2 associated with hypertrophic cardiomyopathy in humans. in Journal of molecular and cellular cardiology 2007
Human Polyclonal JPH2 Primary Antibody for WB - ABIN657368
Yamazaki, Yamazaki, Takeshima: New molecular components supporting ryanodine receptor-mediated Ca(2+) release: roles of junctophilin and TRIC channel in embryonic cardiomyocytes. in Pharmacology & therapeutics 2009
Junctophilin-2 is cleaved by calpain at multiple sites, resulting in dysfunctional junctophilin-2 truncations.
Our data identify a mechanistic link between microtubule densification and T-tubule remodeling and reveal microtubule-mediated JP2 redistribution as a novel mechanism for T-tubule disruption, loss of excitation-contraction coupling, and heart failure.
The S101R mutation may have an effect upon the stability of the JP2 dyad organization with the potential to alter JP2-protein interactions regulating calcium cycling.
This study demonstrates that both JP1 (show JPH1 Antibodies) and JP2 in skeletal muscle undergo Ca2 (show CA2 Antibodies)+-dependent proteolysis by endogenous proteases when the intracellular Ca2 (show CA2 Antibodies)+ is raised within the physiological range for a sustained period
Hypertrophy in skeletal myotubes induced by junctophilin-2 mutant, Y141H, involves an increase in store-operated Ca2 (show CA2 Antibodies)+ entry via Orai1 (show ORAI1 Antibodies).
JP1 (show JPH1 Antibodies) and JP2 can facilitate the assembly of DHPR (show QDPR Antibodies) with other proteins of the excitation-contraction coupling machinery
Loss-of-function mutations in JPH2 leads to cardiac-specific JPH2 deficiency and results in impaired cardiac contractility, causing heart failure and increased mortality.
JPH2 levels are reduced in patients with hypertrophic cardiomyopathy. Reduced JPH2 expression results in reduced excitation-contraction coupling gain as well as altered Ca(2 (show CA2 Antibodies)+) homeostasis, which may be associated with prohypertrophic remodeling.
S165F mutation of junctophilin 2 affects Ca2 (show CA2 Antibodies)+ signalling in skeletal muscle.
this is the first report on JPH2 mutation associated with hypertrophic cardiomyopathy
This molecular rearrangement is compatible with direct inhibition of RyR (show RYR1 Antibodies) opening by junctophilin-2 to intrinsically stabilise the Ca(2 (show CA2 Antibodies)+) signalling properties of the junction and thus the contractile function of the cell.
These results suggested that NCX1 (show SLC8A1 Antibodies) may play an important role in the proper spatial distribution of LTCC and JP2 in T-tubules in the context of pressure-overloading.
overexpression of JP2 provided significantly protective benefits after pressure overload
JPH2 may be critical in anchoring the invaginating sarcolemma to the sarcoplasmic reticulum, thereby enabling the maturation of the transverse tubules network
JP2 deficiency abolished the increase in the density of transverse elements
Our data suggest a novel mechanism by which reduced JPH2-mediated stabilization of RyR2 (show RYR2 Antibodies) due to loss-of-function mutation or reduced JPH2/RyR2 (show RYR2 Antibodies) ratios can promote SR Ca(2 (show CA2 Antibodies)+) leak and atrial arrhythmias
Reducing JP2 expression in ESC-CMs (show Cd2ap Antibodies) resulted in impaired mitochondrial status due to either abnormal cellular Ca(2 (show CA2 Antibodies)+) homeostasis or disturbing of juxtaposition.
Junctional complexes between the plasma membrane and endoplasmic/sarcoplasmic reticulum are a common feature of all excitable cell types and mediate cross talk between cell surface and intracellular ion channels. The protein encoded by this gene is a component of junctional complexes and is composed of a C-terminal hydrophobic segment spanning the endoplasmic/sarcoplasmic reticulum membrane and a remaining cytoplasmic domain that shows specific affinity for the plasma membrane. This gene is a member of the junctophilin gene family. Alternative splicing has been observed at this locus and two variants encoding distinct isoforms are described.
, junctophilin type 2