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Junctophilin

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Junctophilins contribute to the formation of junctional membrane complexes (JMCs) which link the plasma membrane with the endoplasmic or sarcoplasmic reticulum in excitable cells. Provides a structural foundation for functional cross-talk between the cell surface and intracellular calcium release channels. JPH1 contributes to the construction of the skeletal muscle triad by linking the t-tubule (transverse-tubule) and SR (sarcoplasmic reticulum) membranes.

Below are the list of possible Junctophilin products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Junctophilin-1

 Junctophilin-1 ELISA Kit
 Junctophilin-1 Recombinant
 Junctophilin-1 Antibody
Also known as Junctophilin-1 (JP-1) (Junctophilin type 1).
JPH1: Junctophilins contribute to the formation of junctional membrane complexes (JMCs) which link the plasma membrane with the endoplasmic or sarcoplasmic reticulum in excitable cells. Provides a structural foundation for functional cross-talk between the cell surface and intracellular calcium release channels. JPH1 contributes to the
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construction of the skeletal muscle triad by linking the t-tubule (transverse-tubule) and SR (sarcoplasmic reticulum) membranes. Belongs to the junctophilin family.

Protein type: Endoplasmic reticulum; Membrane protein, integral

Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; junctional membrane complex; plasma membrane; sarcoplasmic reticulum; Z disc

Molecular Function: structural constituent of muscle

Biological Process: muscle development
 Jph1 ELISA Kit
 Jph1 Recombinant
 Jph1 Antibody
 Jp1 ELISA Kit
 Jp1 Recombinant
 Jp1 Antibody
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Junctophilin-2

 Junctophilin-2 ELISA Kit
 Junctophilin-2 Recombinant
 Junctophilin-2 Antibody
Also known as Junctophilin-2 (JP-2) (Junctophilin type 2).
JPH2: Junctophilins contribute to the formation of junctional membrane complexes (JMCs) which link the plasma membrane with the endoplasmic or sarcoplasmic reticulum in excitable cells. Provides a structural foundation for functional cross-talk between the cell surface and intracellular calcium release channels. JPH2 is necessary for pr
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oper intracellular Ca(2+) signaling in cardiac myocytes via its involvement in ryanodine receptor-mediated calcium ion release. Contributes to the construction of skeletal muscle triad junctions. Defects in JPH2 are the cause of familial hypertrophic cardiomyopathy type 17 (CMH17). CMH17 is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death. Belongs to the junctophilin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Contractile; Endoplasmic reticulum; Membrane protein, integral

Chromosomal Location of Human Ortholog: 20q13.12

Molecular Function: calcium-release channel activity; phosphatidylinositol 3-phosphate binding; phosphatidylinositol-3,4,5-triphosphate binding; phosphatidylinositol-4,5-bisphosphate binding; phosphatidylinositol-5-phosphate binding; phosphatidylserine binding; protein binding

Biological Process: calcium ion homeostasis

Disease: Cardiomyopathy, Familial Hypertrophic, 17
 JPH2 ELISA Kit
 JPH2 Recombinant
 JPH2 Antibody
 JP2 ELISA Kit
 JP2 Recombinant
 JP2 Antibody
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Junctophilin-3

 Junctophilin-3 ELISA Kit
 Junctophilin-3 Recombinant
 Junctophilin-3 Antibody
Also known as Junctophilin-3 (JP-3) (Junctophilin type 3).
JPH3: Junctophilins contribute to the formation of junctional membrane complexes (JMCs) which link the plasma membrane with the endoplasmic or sarcoplasmic reticulum in excitable cells. Provides a structural foundation for functional cross-talk between the cell surface and intracellular calcium release channels. JPH3 is brain- specific
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and appears to have an active role in certain neurons involved in motor coordination and memory. Defects in JPH3 are the cause of Huntington disease-like type 2 (HDL2). Huntington disease (HD) is a neurodegenerative disorder resulting primarily from the loss of medium spiny projection neurons in the striatum, especially in the caudate nucleus, and, to a lesser extent, atrophy of mesencephalic and cortical structures. The typical clinical picture of HD combines familial adult onset chorea and subcortical dementia that usually begin during the fourth decade of life. Belongs to the junctophilin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Endoplasmic reticulum; Membrane protein, integral

Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; integral to membrane; junctional membrane complex; plasma membrane

Molecular Function: calcium-release channel activity

Biological Process: learning; locomotion; memory; neuromuscular process controlling balance; regulation of neuronal synaptic plasticity
 Jph3 ELISA Kit
 Jph3 Recombinant
 Jph3 Antibody
 Jp3 ELISA Kit
 Jp3 Recombinant
 Jp3 Antibody
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Junctophilin-4

 Junctophilin-4 ELISA Kit
 Junctophilin-4 Recombinant
 Junctophilin-4 Antibody
Also known as Junctophilin-4 (JP-4) (Junctophilin-like 1 protein).
JPH4: Junctophilins contribute to the formation of junctional membrane complexes (JMCs) which link the plasma membrane with the endoplasmic or sarcoplasmic reticulum in excitable cells. Provides a structural foundation for functional cross-talk between the cell surface and intracellular calcium release channels. JPH4 is brain- specific and appears to have an active role in certain neurons involved in motor coordination and memory. Belongs to the junctophilin family.

Protein type: Membrane protein, integral

Chro
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mosomal Location of Human Ortholog: 14q11
 JPH4 ELISA Kit
 JPH4 Recombinant
 JPH4 Antibody
 JPHL1 ELISA Kit
 JPHL1 Recombinant
 JPHL1 Antibody
 KIAA1831 ELISA Kit
 KIAA1831 Recombinant
 KIAA1831 Antibody
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