
Junctophilin 3连接蛋白JPH3抗体
产品名称: Junctophilin 3连接蛋白JPH3抗体
英文名称: Anti-Junctophilin 3 antibody
产品编号: HZ-11083R
产品价格: null
产品产地: 中国/美国
品牌商标: HZbscience
更新时间: 2023-08-17T10:24:20
使用范围: WB,ELISA,IHC-P,IHC-F,IF
- 联系人 : 鲍丽雯
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Junctophilin 3连接蛋白JPH3抗体
产品编号HZ-11083R
英文名称Junctophilin 3
中文名称连接蛋白JPH3抗体
别 名JP-3; JP3; JPH3; JPH3_HUMAN; Junctophilin3; Junctophilin type 3; Junctophilin-3; TNRC22; Trinucleotide repeat-containing gene 22 protein.
说 明 书0.1ml 0.2ml
研究领域心血管 神经生物学 信号转导 细胞粘附分子
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Zebrafish, Sheep,
Junctophilin 3连接蛋白JPH3抗体产品应用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.
分 子 量81kDa
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Junctophilin 3
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Junctophilin 3连接蛋白JPH3抗体PubMedPubMed
产品介绍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 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 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.
Junctophilin 3连接蛋白JPH3抗体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.