| Summary: | 碩士 === 國立臺灣海洋大學 === 生物科技研究所 === 100 === Tcap (also termed telethonin, titin-cap), a 19-kDa protein with a unique β-sheet structure, is exclusively expressed within the Z-disc of adult skeletal and cardiac muscle. Tcap provides binding sites for titin and other Z-disc-associated proteins, and it has also been found that these binding sites of Tcap are lost in the patients of limb-girdle muscular dystrophy type 2G (LGMD 2G). However, the mechanism that Tcap deficiency causes LGMD 2G is currently unknown. Hence, Tcap may be an important therapeutic target in the treatment of patients with limb girdle muscular dystrophy.
In this study, we have established and analyzed the transgenic zebrafish lines expressing various truncated forms of Tcap. On the other hand, we have characterized their skeletal muscle phenotype. The results showed that the abnormality of muscle fibers was definitely observed in
transgenic line Tcap-R3, which lacks the docking sites for titin and other Z-disc-associated proteins. Furthermore, the sarcomere assembly was not affected in Tcap transgenic lines using IHC staining. On the other hand, we also analyzed the hatchability and swimming ability of these transgenic lines at 3 dpf.
In present study, we established transgenic zebrafish lines expressing truncated forms of Tcap, and further provided a novel animal model for studying the disease-causing echanism of LGMD 2G.
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