Cell Signaling Technology

Product Pathways - Ca / cAMP / Lipid Signaling

RyR1 (D4E1) Rabbit mAb #8153

No. Size Price
8153S 100 µl ( 10 western blots ) ¥3,100.00 现货查询 购买询价
8153 carrier free & custom formulation / quantityemail request
Applications Dilution Species-Reactivity Sensitivity MW (kDa) Isotype
W 1:1000 Human,Mouse,Rat, Endogenous ~560 Rabbit IgG
IF-F 1:100

Species cross-reactivity is determined by western blot.

Applications Key: W=Western Blotting, IF-F=Immunofluorescence (Frozen),

Homology

Species predicted to react based on 100% sequence homology: Pig,

Specificity / Sensitivity

RyR1 (D4E1) Rabbit mAb recognizes endogenous levels of total RyR1 protein. This antibody does not cross-react with other ryanodine receptor proteins.

RyR1 (D4E1) Rabbit mAb识别内源性的总RyR1蛋白。该抗体不与其他兰尼碱受体蛋白发生交叉反应。

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Arg830 of human RyR1 protein.

该单克隆抗体通过用合成肽免疫动物制备,该合成肽与人RyR1蛋白精氨酸(830位)附近的残基一样。

IF-F

IF-F

Confocal immunofluorescent analysis of rat skeletal muscle using RyR1 (D4E1) Rabbit mAb (green). Blue pseudocolor = DRAQ5® #4084 (fluorescent DNA dye).

激光共聚焦荧光法检测大鼠骨骼肌组织,使用的抗体为RyR1 (D4E1) Rabbit mAb (绿色).蓝色伪彩色是DNA荧光染料(产品信息为DRAQ5® #4084)。

Western Blotting

Western Blotting

Western blot analysis of extracts from C2C12 and Sol8 cells, undifferentiated (-) or differentiated (+), using RyR1 (D4E1) Rabbit mAb.

Western blot方法检测C2C12和Sol8细胞提取物,细胞未分化(-)或已分化(+),使用的抗体为RyR1 (D4E1) Rabbit mAb.

Background

Ryanodine receptors (RyRs) are large (>500 kDa), intracellular calcium channels found in the sarcoplasmic/endoplasmic reticulum membrane and are responsible for the release of Ca2+ from intracellular stores in excitable cells, such as muscle and neurons. RyRs exist as three mammalian isoforms (RyR1-3), all of which form homotetramers regulated by phosphorylation and/or direct or indirect interaction with a variety of proteins (L-type calcium channels, PKA, FKBP12/12.6, CaMKII, calmodulin, calsequestrin, junctin, and triadin) and ions (Mg2 + and Ca2 +). Regulation of the RyR channel by protein modulators occurs within the large cytoplasmic domain, whereas the carboxy-terminal portion of the protein forms the ion-binding and conducting pore (1,2). RyR1 and RyR2 are predominantly expressed in skeletal and cardiac muscle, respectively, where they localize exclusively to the sarcoplasmic reticulum (SR) and facilitate calcium-mediated communication between transverse-tubules and sarcoplasmic reticulum. Contraction of skeletal muscle is triggered by release of calcium ions from the SR following depolarization of T-tubules. Research studies have shown that defects in RyR1 are the cause of malignant hyperthermia susceptibility type 1 (MHS1), central core disease of muscle (CCD), multiminicore disease with external ophthalmoplegia, and congenital myopathy with fiber-type disproportion (CFTD), each of which is manifested by defects in muscle function, metabolism, and development (2). Investigators have shown that defects in RyR2 are the cause of familial arrhythmogenic right ventricular dysplasia type 2 (ARVD2) and catecholaminergic polymorphic ventricular tachycardia type 1 (CPVT1), both of which are implicated in sudden death syndromes as a result of electrical instability and degeneration of the ventricular myocardium or stress-induced ventricular tachycardia (2). Despite low levels of expression in skeletal and smooth muscle, RyR3 is the dominant isoform in neuronal cells (hippocampal neurons, thalamus, Purkinje cells) and has been implicated in synaptic plasticity, dendritic spine remodeling, and spatial memory formation (3). The role of RyR3 in neuronal function has been substantiated by mice lacking RyR3, which demonstrate normal motor function, but possess numerous behavioral and social defects (4).

兰尼碱受体(RyRs)是在肌浆/内质网膜中发现的大分子量(>500 kDa)细胞内钙通道,在可兴奋性细胞中负责细胞内存储的Ca2 +的释放。在哺乳动物中,兰尼碱受体存在三种亚型(RyR1,RyR2,RyR3),所有这些形成同源四聚体,由磷酸化作用调节,和/或直接或间接与多种蛋白(L-型钙离子通道, PKA, FKBP12/12.6, CaMKII, 钙调蛋白, 肌集钙蛋白, 接头蛋白和三合蛋白)和离子(Mg2 +和Ca2 +)相互作用。通过蛋白调节器,RyR通道的调节发生在大的胞质结构域,在那里蛋白质的羧基末端部分形成离子结合和传导孔(1,2)。RyR1和RyR2主要分别表达在骨骼肌和心肌,完全定位在肌浆网(SR),方便横管和肌浆网钙离子介导的沟通。骨骼肌收缩是在T-管去极化后,由SR释放钙离子触发的。调查研究表明,RyR1缺陷是恶性高热易感性1型(MHS1)、核心肌肉疾病(CCD)、多微小轴空病与眼外肌麻痹、先天性肌病、纤维型比例失调(CFTD)的原因,每个疾病都证明是肌肉功能、代谢和发生缺陷(2)。研究已显示,RyR2的缺陷是家族性致心律失常性右心室发育不良2型(ARVD2)和儿茶酚胺的多晶型室性心动过速1型(CPVT1)的原因,这两个都与猝死综合征心室肌或应力诱发室性心动过速有关,是电不稳定和变性的结果(2)。尽管RyR3在骨骼肌和平滑肌中低水平表达,但其在神经细胞(丘脑、海马神经元、浦肯野细胞)中是占主导地位的异构体,并且与突触可塑性、树突棘的重塑和空间记忆的形成有关(3)。已经通过RyR3缺乏小鼠证实RYR3的神经元功能作用,表明小鼠运动功能正常,但拥有很多行为和社会缺陷(4)。

  1. Lanner, J.T. et al. (2010) Cold Spring Harb Perspect Biol 2, a003996.
  2. Betzenhauser, M.J. and Marks, A.R. (2010) Pflugers Arch 460, 467-80.
  3. Adasme, T. et al. (2011) Proc Natl Acad Sci U S A 108, 3029-34.
  4. Matsuo, N. et al. (2009) Front Behav Neurosci 3, 3.

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For Research Use Only. Not For Use In Diagnostic Procedures.

DRAQ5 is a registered trademark of Biostatus Limited.

Cell Signaling Technology is a trademark of Cell Signaling Technology, Inc.

Cell Signaling Technology® is a trademark of Cell Signaling Technology, Inc.

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