连接蛋白闯笔贬3抗体
规格：1尘驳/1尘濒
英文名： Junctophilin 3
别名： JP-3; JP3; JPH3; JPH3_HUMAN; Junctophilin3; Junctophilin type 3; Junctophilin-3; TNRC22; Trinucleotide repeat-containing gene 22 protein.
分子量： 81kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Junctoph
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Zebrafish, Sheep,
细胞定位：细胞浆 细胞膜
连接蛋白闯笔贬3抗体产物介绍：background: Junctophilins are components of the junctional complexes between plasma membranes and endoplasmic or sarcoplasmic reticulums present in all excitable cells. Junctophilins contain a cytoplasmic domain which binds to the plasma membrane, as well as an ER/SR membrane spanning hydrophobic C-terminal segment. The three subtypes in this family are Junctophilin-1, -2 and -3. Junctophilin-1 is predominantly expressed in skeletal muscle, but is also expressed at low levels in heart. Junctophilin-2 is expressed in heart and skeletal muscle. Mutant mice lacking the Jph2 gene exhibit embryonic lethality and possess cardiac myocytes that express abnormal calcium transients. Junctophilin-3 is expressed in brain. The JPH3 alternatively spliced exon 2A has been suggested as a site for CTG repeat expansion leading to a连接蛋白闯笔贬3抗体 Huntington disease-like autosomal dominant disorder. Function: Contributes to the stabilization of the junctional membrane complexes, which are common to excitable cells and mediate cross-talk between cell surface and intracellular ion channels. Probably acts by anchoring the plasma membrane and endoplasmic reticulum. May play an active role in certain neurons involved in motor coordination. Subcellular Location: Cell membrane. Endoplasmic reticulum membrane. Localized predominantly on the plasma membrane. The transmembrane domain is anchored in endoplasmic reticulum membrane, while the N-terminal part associates with the plasma membrane. Tissue Specificity: Specifically expressed in brain. DISEASE: Defects in JPH3 are the cause of Huntington disease-like type 2 (HDL2) [MIM:606438]. Huntington disease (HD) is a neurodegenerative 连接蛋白闯笔贬3抗体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. Similarity: Belongs to the junctophilin family. Contains 8 MORN repeats. Database links: UniProtKB/Swiss-Prot: Q8WXH2.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
连接蛋白闯笔贬3抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：心血管 神经生物学 信号转导 细胞粘附分子
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid