Emery dreifuss muscular dystrophy(EDMD) is a rare genetic syndrome consisting of tendon retractions,progressive muscle atrophy and cardiac involvement.We report a case of an obese patient affected by the familial X-li...Emery dreifuss muscular dystrophy(EDMD) is a rare genetic syndrome consisting of tendon retractions,progressive muscle atrophy and cardiac involvement.We report a case of an obese patient affected by the familial X-linked form in which a pericallosal lipoma was found during investigation for a suspected acute vasculopathy.To date,EDMD has never been associated with cerebral lipomas and the X-linked form was never considered to be involved in lipodystrophic syndromes or non-muscular conditions.Our case confirms the variable expressivity of the disease and suggests a possible role of Emerin in the intranuclear regulation of signals for adipocyte cell differentiation.展开更多
Based on the hydrographic data in austral summer during the 22nd Antarctic Expedition of China(2005/2006),some features can be found about the northern margin of Emery ice shelf as follows.The heat content in the surf...Based on the hydrographic data in austral summer during the 22nd Antarctic Expedition of China(2005/2006),some features can be found about the northern margin of Emery ice shelf as follows.The heat content in the surface layer(0-50 m) at the eastern end and the western end of the ice-shelf margin is much higher than that at the middle.The upper mixing-layer depth and the seasonal thermocline depth at the middle of the ice-shelf northern margin are much shallower than those at the both ends.However there is much less difference between the middle and the ends in the bottom layer.The remote sensing photos show that the inhomogeneity in the surface-layer water is closely related to the spatial distribution of the floes and polynia in the area.展开更多
We report a striking abundance of rimmed vacuoles in two brothers with X- li nked Emery- Dreifuss muscular dystrophy (X- EDMD) confirmed by the absence of emerin at the muscular nuclear envelope and by genetic analysi...We report a striking abundance of rimmed vacuoles in two brothers with X- li nked Emery- Dreifuss muscular dystrophy (X- EDMD) confirmed by the absence of emerin at the muscular nuclear envelope and by genetic analysis showing a new 2 - bp deletion in exon 6 of the STA gene at the Xq28 region. Immunohistochemical analysis of the vacuoles revealed expression of dystrophin but not of merosin i n the sarcolemma of rimmed vacuoles and absence of amyloid and membrane attack c omplex (MAC) deposition either in vacuoles or muscle fibers. The presence of rim med vacuoles can be a histopathological finding in X- EDMD, and the diagnosis s hould not be excluded in clinically well- defined EDMD patients because of this finding.展开更多
In this paper, the equilibrium properties of spin-1 Blume–Emery–Griffiths model are studied by using constant-coupling approximation. The dipolar and quadrupolar order parameters, the stable, metastable and unstable...In this paper, the equilibrium properties of spin-1 Blume–Emery–Griffiths model are studied by using constant-coupling approximation. The dipolar and quadrupolar order parameters, the stable, metastable and unstable states and free energy of the model are investigated. The states are defined in terms of local minima of the free energy of system. The numerical calculations are presented for several values of exchange interactions on the simple cubic lattice with q = 6.展开更多
文摘Emery dreifuss muscular dystrophy(EDMD) is a rare genetic syndrome consisting of tendon retractions,progressive muscle atrophy and cardiac involvement.We report a case of an obese patient affected by the familial X-linked form in which a pericallosal lipoma was found during investigation for a suspected acute vasculopathy.To date,EDMD has never been associated with cerebral lipomas and the X-linked form was never considered to be involved in lipodystrophic syndromes or non-muscular conditions.Our case confirms the variable expressivity of the disease and suggests a possible role of Emerin in the intranuclear regulation of signals for adipocyte cell differentiation.
文摘Based on the hydrographic data in austral summer during the 22nd Antarctic Expedition of China(2005/2006),some features can be found about the northern margin of Emery ice shelf as follows.The heat content in the surface layer(0-50 m) at the eastern end and the western end of the ice-shelf margin is much higher than that at the middle.The upper mixing-layer depth and the seasonal thermocline depth at the middle of the ice-shelf northern margin are much shallower than those at the both ends.However there is much less difference between the middle and the ends in the bottom layer.The remote sensing photos show that the inhomogeneity in the surface-layer water is closely related to the spatial distribution of the floes and polynia in the area.
文摘We report a striking abundance of rimmed vacuoles in two brothers with X- li nked Emery- Dreifuss muscular dystrophy (X- EDMD) confirmed by the absence of emerin at the muscular nuclear envelope and by genetic analysis showing a new 2 - bp deletion in exon 6 of the STA gene at the Xq28 region. Immunohistochemical analysis of the vacuoles revealed expression of dystrophin but not of merosin i n the sarcolemma of rimmed vacuoles and absence of amyloid and membrane attack c omplex (MAC) deposition either in vacuoles or muscle fibers. The presence of rim med vacuoles can be a histopathological finding in X- EDMD, and the diagnosis s hould not be excluded in clinically well- defined EDMD patients because of this finding.
文摘In this paper, the equilibrium properties of spin-1 Blume–Emery–Griffiths model are studied by using constant-coupling approximation. The dipolar and quadrupolar order parameters, the stable, metastable and unstable states and free energy of the model are investigated. The states are defined in terms of local minima of the free energy of system. The numerical calculations are presented for several values of exchange interactions on the simple cubic lattice with q = 6.
