Selective Vacuolar Degeneration in Dystrophin-Deficient Canine Purkinje Fibers Despite Preservation of Dystrophin-Associated Proteins With Overexpression of Dp71
Author(s) -
Nobuyuki Urasawa,
Michiko Wada,
Noboru Machida,
Katsutoshi Yuasa,
Yoshiki Shimatsu,
Yoshito Wakao,
Shigeki Yuasa,
Toshiaki Sano,
Ikuya aka,
Akinori Nakamura,
Shin’ichi Takeda
Publication year - 2008
Publication title -
circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.795
H-Index - 607
eISSN - 1524-4539
pISSN - 0009-7322
DOI - 10.1161/circulationaha.107.739326
Subject(s) - dystrophin , sarcolemma , purkinje cell , purkinje fibers , duchenne muscular dystrophy , pathology , medicine , degeneration (medical) , desmin , muscular dystrophy , utrophin , microbiology and biotechnology , biology , cerebellum , myocyte , immunohistochemistry , electrophysiology , vimentin
Respiratory support therapy significantly improves life span in patients with Duchenne muscular dystrophy; cardiac-related fatalities, including lethal arrhythmias, then become a crucial issue. It is therefore important to more thoroughly understand cardiac involvement, especially pathology of the conduction system, in the larger Duchenne muscular dystrophy animal models such as dystrophic dogs.
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