JPH3, Polyclonal Antibody

JPH3; JP3; HDL2; JP-3; TNRC22; CAGL237; JP3; Junctophilin-3; Junctophilin type 3; junctophilin 3

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

JPH3 Antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL (lot specific)

JPH3 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Small volumes of anti-JPH3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

JPH3 Antibody: Junctional complexes between the plasma membrane (PM) and endoplasmic/sarcoplasmic reticulum (ER/SR) are a common feature of all excitable cell types and mediate cross talk between cell surface and intracellular ion channels. Junctophilins (JPs) are important components of the junctional complexes. JPs are composed of a carboxy-terminal hydrophobic segment spanning the ER/SR membrane and a remaining cytoplasmic domain that shows specific affinity for the PM. Four JPs have been identified as tissue-specific subtypes derived from different genes: JPH1 is expressed in skeletal muscle, JPH2 is detected throughout all muscle cell types, and JPH3 and JPH4 are predominantly expressed in the brain. In the CNS, both JPH3 and JPH4 are expressed throughout neural sites and contribute to the subsurface cistern formation in neurons. Mice lacking both JPH3 and JPH4 subtypes exhibit serious symptoms such as impaired learning and memory and are accompanied by abnormal nervous functions. A repeat expansion in JPH3 is associated with Huntington disease-like 2. At least two isoforms of JPH3 are known to exist.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

JPH3 antibody can be used for detection of JPH3 by Western blot at 1 mug/mL. Antibody can also be used for immunohistochemistry starting at 2.5 mug/mL. For immunofluorescence start at 20 mug/mL.

Western blot analysis of JPH3 in Daudi cell lysate with JPH3 antibody at 1 μg/mL in (A) the absence and (B) the presence of blocking peptide.

Immunohistochemistry of JPH3 in human brain tissue with JPH3 antibody at 2.5 μg/mL.

Immunofluorescence of JPH3 in Human Brain cells with JPH3 antibody at 20 μg/mL.

19,766 Da

junctophilin 3

junctophilin-3; junctophilin type 3; trinucleotide repeat containing 22

Junctophilin-3

JP3; TNRC22; JP-3  [Similar Products]

JPH3_HUMAN

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. CAG/CTG repeat expansions at the Huntington's disease (HD)-like 2 locus have been identified in this gene, which is a member of the junctophilin gene family. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Oct 2012]

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 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 ***** 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

Chromosomal Location of Human Ortholog: 16q24.3

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

Molecular Function: calcium-release channel activity

Biological Process: learning; locomotion; neuromuscular process controlling balance; memory; regulation of neuronal synaptic plasticity

Disease: Huntington Disease-like 2

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