Muscle & nerve · 2017
Transforming growth factor-β signaling is upregulated in sporadic inclusion body myositis.
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Noda Seiya, Koike Haruki, Maeshima Shinya, Nakanishi Hirotaka, Iijima Masahiro, Matsuo Koji, Kimura Seigo, Katsuno Masahisa, Sobue Gen
Аннотация
INTRODUCTION: In this study we aimed to determine whether transforming growth factor-β (TGF-β) signaling is dysregulated in sporadic inclusion body myositis (sIBM) muscle samples.
METHODS: We examined TGF-β signaling markers in muscle samples from 24 sIBM patients and compared them with those from 10 dermatomyositis (DM) patients using immunohistochemistry and Western blot analyses.
RESULTS: Compared with the DM muscle fibers, the sIBM muscle fibers exhibited greater TGF-β, TGF-β receptor type I (TβRI), and TGF-β receptor type II (TβRII) immunoreactivity in the cytoplasm, as well as greater phosphorylated Smad2 (pSmad2) immunoreactivity in the myonuclei. The signal intensities of TGF-β, TβRI, and TβRII immunoreactivity correlated significantly with muscle fiber cross-sectional areas. Western blot analyses indicated higher expression levels of TGF-β, TβRI, TβRII, and pSmad2 in the sIBM muscle samples than in the DM muscle samples.
CONCLUSIONS: These data indicate that upregulation of TGF-β signaling may be an important molecular event in sIBM. Muscle Nerve 55: 741-747, 2017.
METHODS: We examined TGF-β signaling markers in muscle samples from 24 sIBM patients and compared them with those from 10 dermatomyositis (DM) patients using immunohistochemistry and Western blot analyses.
RESULTS: Compared with the DM muscle fibers, the sIBM muscle fibers exhibited greater TGF-β, TGF-β receptor type I (TβRI), and TGF-β receptor type II (TβRII) immunoreactivity in the cytoplasm, as well as greater phosphorylated Smad2 (pSmad2) immunoreactivity in the myonuclei. The signal intensities of TGF-β, TβRI, and TβRII immunoreactivity correlated significantly with muscle fiber cross-sectional areas. Western blot analyses indicated higher expression levels of TGF-β, TβRI, TβRII, and pSmad2 in the sIBM muscle samples than in the DM muscle samples.
CONCLUSIONS: These data indicate that upregulation of TGF-β signaling may be an important molecular event in sIBM. Muscle Nerve 55: 741-747, 2017.
Предметные рубрики MeSH
DermatomyositisFemaleHumansMaleMuscle, SkeletalMyositis, Inclusion BodyPhosphorylationProtein Serine-Threonine KinasesReceptor, Transforming Growth Factor-beta Type IReceptor, Transforming Growth Factor-beta Type IIReceptors, Transforming Growth Factor betaSignal TransductionSmad2 ProteinTransforming Growth Factor betaUp-Regulation