Article
Pathological evaluation of rats carrying in-frame mutations in the dystrophin gene: a new model of Becker muscular dystrophy.
Disease models & mechanisms - 28 Sept 2020
Teramoto Naomi, Sugihara Hidetoshi, Yamanouchi Keitaro, Nakamura Katsuyuki, Kimura Koichi, Okano Tomoko, Shiga Takanori, Shirakawa Taku, Matsuo Masafumi, Nagata Tetsuya, Daimon Masao, Matsuwaki Takashi, Nishihara Masugi
Abstract excerpt
Dystrophin, encoded by the DMD gene on the X chromosome, stabilizes the sarcolemma by linking the actin cytoskeleton with the dystrophin-glycoprotein complex (DGC). In-frame mutations in DMD cause a milder form of X-linked muscular dystrophy, called Becker muscular dystrophy (BMD), characterized by the reduced expression of truncated dystrophin. So far, no animal model with in-frame mutations in Dmd has been...
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