预约演示

更新于：2025-05-07

telethonin

更新于：2025-05-07

基本信息

别名

CMD1N、LGMD2G、T-cap

+ [5]

简介

Muscle assembly regulating factor. Mediates the antiparallel assembly of titin (TTN) molecules at the sarcomeric Z-disk.

关联

项与 telethonin 相关的药物

Limb Girdle Muscular Dystrophy 2g(CRD)

靶点

telethonin

作用机制

telethonin 刺激剂

在研机构

Cure Rare Disease, Inc.

原研机构

Cure Rare Disease, Inc.

在研适应症

肢带型肌营养不良2G型

非在研适应症-

最高研发阶段临床前

首次获批国家/地区-

首次获批日期1800-01-20

rAAV.TCAP

靶点

telethonin

作用机制

telethonin modulators

在研机构

UMass Chan Medical School [+1]

原研机构

UMass Chan Medical School [+1]

在研适应症

肢带型肌营养不良2G型

非在研适应症-

最高研发阶段临床前

首次获批国家/地区-

首次获批日期1800-01-20

100 项与 telethonin 相关的临床结果

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100 项与 telethonin 相关的转化医学

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0 项与 telethonin 相关的专利（医药）

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267

项与 telethonin 相关的文献（医药）

2025-06-01·Food Chemistry

Structural evaluation and formation regularities of garlic clove-like meat in prefabricated grass carp

Article

作者: Tian, Yiling ; Zhang, Yuting ; Liu, Ru ; Rong, Jianhua ; Xiong, Shanbai ; Yang, Qin ; Zhao, Yongqiang ; Song, Liang ; Xie, Junhong ; Xiong, Jian ; Hu, Yang

Moderately marinated fish meat forms a garlic clove-like structure after cooking. However, this structure's formation mechanism and influencing factors are unclear. Therefore, this study explored the changes in protein components and potential protein markers during the formation of garlic clove-like structure. Results showed that the contents of free hydroxyproline, lysylpyridinoline, and hydroxylysylpyridinoline first increased and then decreased, the contents of decorin and elastin decreased, and TCA-soluble peptides increased slightly during the formation process of garlic clove-like structure. The Z-disk, M-line, and H bands in the myofibrils disappeared, and the myofibrils were interconnected. This was attributed to the effect of endogenous proteases (MMP, calpain, etc.) on myofibrils, collagen fibers, and proteoglycans. Proteomics data showed that the abundance of type I collagen chains, myosin, actin, and telethonin increased while proteoglycans decreased. Among them, telethonin on the Z-disk can be used as a potential protein biomarker for the formation of garlic clove-like structure.

2025-02-24·JCI Insight

Constitutive deletion of the obscurin-Ig58/59 domains induces atrial remodeling and Ca2+-based arrhythmogenesis

Article

作者: Boyman, Liron ; Greiser, Maura ; Brong, Annie ; Joca, Humberto C ; Grogan, Alyssa ; Ward, Christopher W ; Kaplan, Aaron D ; Kontrogianni-Konstantopoulos, Aikaterini

Obscurin is a giant protein that coordinates diverse aspects of striated muscle physiology. Obscurin immunoglobulin domains 58/59 (Ig58/59) associate with essential sarcomeric and Ca2+ cycling proteins. To explore the pathophysiological significance of Ig58/59, we generated the Obscn-ΔIg58/59 mouse model, expressing obscurin constitutively lacking Ig58/59. Males in this line develop atrial fibrillation by 6 months, with atrial and ventricular dilation by 12 months. As Obscn-ΔIg58/59 left ventricles at 6 months exhibit no deficits in sarcomeric ultrastructure or Ca2+ signaling, we hypothesized that susceptibility to arrhythmia may emanate from the atria. Ultrastructural evaluation of male Obscn-ΔIg58/59 atria uncovered prominent Z-disk streaming by 6 months and further misalignment by 12 months. Relatedly, isolated Obscn-ΔIg58/59 atrial cardiomyocytes exhibited increased Ca2+ spark frequency and age-specific alterations in Ca2+ cycling dynamics, coinciding with arrhythmia onset and progression. Quantitative analysis of the transverse-axial tubule (TAT) network using super-resolution microscopy demonstrated significant TAT depletion in Obscn-ΔIg58/59 atria. These structural and Ca2+ signaling deficits were accompanied by age-specific alterations in the expression or phosphorylation of T-cap protein, which links transverse tubules to Z-disks, and junctophilin 2, which connects transverse tubules to the sarcoplasmic reticulum. Collectively, our work establishes the Obscn-ΔIg58/59 model as a reputable genetic model for atrial cardiomyopathy and provides mechanistic insights into atrial fibrillation and remodeling.

2025-01-06·GigaScience

Exploring the cellular and molecular basis of murine cardiac development through spatiotemporal transcriptome sequencing

Article

作者: Shen, Xunan ; Wang, Jizheng ; Wang, Yue ; Li, Qingsong ; Kang, Jingmin ; Liu, Fuyan ; Wu, Renhua ; Liu, Jie ; Song, Lei ; Liu, Xin ; Liu, Peng ; Wang, Ninghe ; Luo, Xujiao ; Du, Lin

AbstractBackgroundSpatial transcriptomics is a powerful tool that integrates molecular data with spatial information, thereby facilitating a deeper comprehension of tissue morphology and cellular interactions. In our study, we utilized cutting-edge spatial transcriptome sequencing technology to explore the development of the mouse heart and construct a comprehensive spatiotemporal cell atlas of early murine cardiac development.ResultsThrough the analysis of this atlas, we elucidated the spatial organization of cardiac cellular lineages and their interactions during the developmental process. Notably, we observed dynamic changes in gene expression within fibroblasts and cardiomyocytes. Moreover, we identified critical genes, such as Igf2, H19, and Tcap, as well as transcription factors Tcf12 and Plagl1, which may be associated with the loss of myocardial regeneration ability during early heart development. In addition, we successfully identified marker genes, like Adamts8 and Bmp10, that can distinguish between the left and right atria.ConclusionOur study provides novel insights into murine cardiac development and offers a valuable resource for future investigations in the field of heart research, highlighting the significance of spatial transcriptomics in understanding the complex processes of organ development.

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