Estrada Francisco J, Mornet Dominique, Rosas-Vargas Haydeé, Angulo Alexandra, Hernández Manuel, Becker Viola, Rendón Alvaro, Ramos-Kuri Manuel, Coral-Vázquez Ramón M
Unidad de Investigación Médica en Genética Humana, Hospital de Pediatría, Centro Médico Nacional Siglo XXI-IMSS, Av. Cuauhtémoc 330, Col. Doctores, C.P. 06725 México, DF, Mexico.
Biochem Biophys Res Commun. 2006 Feb 17;340(3):865-71. doi: 10.1016/j.bbrc.2005.12.083. Epub 2005 Dec 27.
The sarcoglycan-sarcospan complex (alpha-, beta-, gamma-, delta-, epsilon-, and zeta-SG-SSPN), a component of the dystrophin-associated glycoprotein complex (DAGC), is located at the sarcolemma of muscle fibers where it contributes to maintain cell integrity during contraction-relaxation cycles; gamma- and delta-SG are also expressed in the sarcoplasmic reticulum (SR). In this study, we report the identification of a novel isoform of murine delta-SG produced by alternative splicing that we named delta-SG3. This isoform is present at transcript level in several tissues, with its highest expression in skeletal and cardiac muscle. The delta-SG3 protein lacks the last 122 amino acids at the C-terminal, which are replaced by 10 new amino acids (EGFLNMQLAG). Interestingly, double immunofluorescence analysis for delta-SG3 and the dihydropyridine receptor (DHPR) shows a close localization of these two proteins. We propose the subcellular distribution of this novel delta-SG3 isoform at the SR and its involvement in intracellular calcium concentration regulation.
肌聚糖 - 肌膜蛋白复合体(α -、β -、γ -、δ -、ε - 和ζ - 肌聚糖 - 肌膜蛋白)是肌营养不良蛋白相关糖蛋白复合体(DAGC)的一个组成部分,位于肌纤维的肌膜上,在收缩 - 舒张周期中有助于维持细胞完整性;γ - 和δ - 肌聚糖也在肌浆网(SR)中表达。在本研究中,我们报告了通过可变剪接产生的小鼠δ - 肌聚糖的一种新型异构体的鉴定,我们将其命名为δ - SG3。这种异构体在转录水平存在于多种组织中,在骨骼肌和心肌中表达最高。δ - SG3蛋白在C末端缺少最后122个氨基酸,被10个新氨基酸(EGFLNMQLAG)取代。有趣的是,对δ - SG3和二氢吡啶受体(DHPR)的双重免疫荧光分析显示这两种蛋白紧密定位。我们提出这种新型δ - SG3异构体在肌浆网的亚细胞分布及其参与细胞内钙浓度调节。