Article
Pharmacological activation of SERCA ameliorates dystrophic phenotypes in dystrophin-deficient mdx mice.
Human molecular genetics - 31 May 2021
Nogami Ken'ichiro, Maruyama Yusuke, Sakai-Takemura Fusako, Motohashi Norio, Elhussieny Ahmed, Imamura Michihiro, Miyashita Satoshi, Ogawa Megumu, Noguchi Satoru, Tamura Yuki, Kira Jun-Ichi, Aoki Yoshitsugu, Takeda Shin'ichi, Miyagoe-Suzuki Yuko
Abstract excerpt
Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder characterized by progressive muscular weakness because of the loss of dystrophin. Extracellular Ca2+ flows into the cytoplasm through membrane tears in dystrophin-deficient myofibers, which leads to muscle contracture and necrosis. Sarco/endoplasmic reticulum Ca2+-ATPase (SERCA) takes up cytosolic Ca2+ into the sarcoplasmic reticulum, but its...
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