CAGL237; FLJ44707; HDL2; JP-3; JP3; TNRC22

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

Polyclonal

IgG

Rabbit

Antigen affinity purification

Store at -20 degree C. Avoid freeze / thaw cycles

Manufactured in an ISO 9001:2015 Certified Laboratory.

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.

ELISA (EIA)

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: Membrane protein, integral; Endoplasmic reticulum

Chromosomal Location of Human Ortholog: 16q24.3

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