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排序方式: 共有163条查询结果,搜索用时 15 毫秒
1.
Kimura T Nakamori M Lueck JD Pouliquin P Aoike F Fujimura H Dirksen RT Takahashi MP Dulhunty AF Sakoda S 《Human molecular genetics》2005,14(15):2189-2200
Myotonic dystrophy type 1 (DM1) is a debilitating multisystemic disorder caused by a CTG repeat expansion in the DMPK gene. Aberrant splicing of several genes has been reported to contribute to some symptoms of DM1, but the cause of muscle weakness in DM1 and elevated Ca2+ concentrations in cultured DM muscle cells is unknown. Here, we investigated the alternative splicing of mRNAs of two major proteins of the sarcoplasmic reticulum, the ryanodine receptor 1 (RyR1) and sarcoplasmic/endoplasmic reticulum Ca2+-ATPase (SERCA) 1 or 2. The fetal variants, ASI(-) of RyR1 which lacks residue 3481-3485, and SERCA1b which differs at the C-terminal were significantly increased in skeletal muscles from DM1 patients and the transgenic mouse model of DM1 (HSA(LR)). In addition, a novel variant of SERCA2 was significantly decreased in DM1 patients. The total amount of mRNA for RyR1, SERCA1 and SERCA2 in DM1 and the expression levels of their proteins in HSA(LR) mice were not significantly different. However, heterologous expression of ASI(-) in cultured cells showed decreased affinity for [3H]ryanodine but similar Ca2+ dependency, and decreased channel activity in single-channel recording when compared with wild-type (WT) RyR1. In support of this, RyR1-knockout myotubes expressing ASI(-) exhibited a decreased incidence of Ca2+ oscillations during caffeine exposure compared with that observed for myotubes expressing WT-RyR1. We suggest that aberrant splicing of RyR1 and SERCA1 mRNAs might contribute to impaired Ca2+ homeostasis in DM1 muscle. 相似文献
2.
Harutoshi Asakawa Gen Sazaki Ken Nagashima Shunichi Nakatsubo Yoshinori Furukawa 《Proceedings of the National Academy of Sciences of the United States of America》2016,113(7):1749-1753
Surfaces of ice are covered with thin liquid water layers, called quasi-liquid layers (QLLs), even below their melting point (0 °C), which govern a wide variety of phenomena in nature. We recently found that two types of QLL phases appear that exhibit different morphologies (droplets and thin layers) [Sazaki G. et al. (2012) Proc Natl Acad Sci USA 109(4):1052−1055]. However, revealing the thermodynamic stabilities of QLLs remains a longstanding elusive problem. Here we show that both types of QLLs are metastable phases that appear only if the water vapor pressure is higher than a certain critical supersaturation. We directly visualized the QLLs on ice crystal surfaces by advanced optical microscopy, which can detect 0.37-nm-thick elementary steps on ice crystal surfaces. At a certain fixed temperature, as the water vapor pressure decreased, thin-layer QLLs first disappeared, and then droplet QLLs vanished next, although elementary steps of ice crystals were still growing. These results clearly demonstrate that both types of QLLs are kinetically formed, not by the melting of ice surfaces, but by the deposition of supersaturated water vapor on ice surfaces. To our knowledge, this is the first experimental evidence that supersaturation of water vapor plays a crucially important role in the formation of QLLs.Ice is one of the most abundant materials on Earth, and its surfaces are covered with thin liquid water layers even below their melting point (0 °C) (1–4). Such thin liquid water layers are called “quasi-liquid layers” (QLLs). Because QLLs govern the surface properties of ice just below the melting point, it is well acknowledged that surface melting of ice governs a wide variety of phenomena, such as electrification of thunderclouds (4, 5), regelation (4, 6), frost heave (4, 7), conservation of foods, ice skating (1, 8), preparation of a snowman (1), and growth of ice crystals (2, 4). Therefore, it is essential to understand the surface melting of ice crystals at the molecular level.After Michael Faraday proposed the existence of QLLs in 1842 (1), many studies experimentally confirmed the formation of QLLs by various methods (Measurement method Reference First author (experimental condition) Proton channeling (24, 25) G (S) Proton backscattering (26, 27) F (U) Ellipsometry (23, 28) F (U), B (E) X-ray diffraction (29) K (E) Glancing-angle X-ray scattering (30, 31) D (U) Quasi-elastic neutron scattering (32) M (E) Photoelectron spectroscopy (33, 34) N (E), B (E) NMR (35, 36) K (E), M (U) Optical microscopy (37, 38) E (S), G (S) Optical displacement sensor (39) K (U) Infrared spectroscopy (40) S (S) Sum-frequency vibrational spectroscopy (41, 42) W (E) Atomic force microscopy (43–46) B (S), D (U), S (U, E, S), P (E)