Charcot-Marie-Tooth disease(CMT)is a hereditary peripheral neuropathy causing muscle weakness/wasting and sensory dysfunction predominantly in limb extremities.CMT patients display gait abnormalities,foot deformities,...Charcot-Marie-Tooth disease(CMT)is a hereditary peripheral neuropathy causing muscle weakness/wasting and sensory dysfunction predominantly in limb extremities.CMT patients display gait abnormalities,foot deformities,loss of sensation and decreased/absent deep tendon reflexes,with motor symptoms usually being more prominent than sensory.展开更多
Muscle regeneration after damage or during myopathies requires a fine cooperation between myoblast proliferation and myogenic differentiation.A growing body of evidence suggests that microRNAs play critical roles in m...Muscle regeneration after damage or during myopathies requires a fine cooperation between myoblast proliferation and myogenic differentiation.A growing body of evidence suggests that microRNAs play critical roles in myocyte proliferation and differentiation transcriptionally.However,the molecular mechanisms underlying the orchestration are not fully understood.Here,we showed that miR-130b is able to repress myoblast proliferation and promote myogenic differentiation via targeting Sp1 transcription factor.Importantly,overexpression of miR-130b is capable of improving the recovery of damaged muscle in a freeze injury model.Moreover,miR-130b expression is declined in the muscle of muscular dystrophy patients.Thus,these results indicated that miR-130b may play a role in skeletal muscle regeneration and myopathy progression.Together,our findings suggest that the miR-130b/Sp1 axis may serve as a potential therapeutic target for the treatment of patients with muscle damage or severe myopathies.展开更多
目的系统调查文艺复兴时期的伟大画家如何在作品中呈现幼年耶稣的巴宾斯基征(Babinski sign)。设计观察性研究背景通过已发表的编目和谷歌。搜索大量公元1400-1550年期间弗菜芒画派(Flemish).莱茵面派(Rhenish)和意大利学院源两家绘制...目的系统调查文艺复兴时期的伟大画家如何在作品中呈现幼年耶稣的巴宾斯基征(Babinski sign)。设计观察性研究背景通过已发表的编目和谷歌。搜索大量公元1400-1550年期间弗菜芒画派(Flemish).莱茵面派(Rhenish)和意大利学院源两家绘制的幼年耶穌画作。研究样本302幅文艺复兴时期描绘幼年基督的画作(来自19位画家)。主要结局指标巴宾斯基征的定义为婴儿大脚趾张开幅度超过30度。还应注意来自足底的朝激。结果在302幅绘面中,有90幅绘画(占比约30%)中出现了明显向上张开的脚趾。在由Rogier van der Weyden,Hans Menling,Marin Schongouer和Mathias Grinewald创作的幼年基督的作品中,有超过60%出现了巴宾斯基征。甚至在3幅西中,罌儿的两只脚都出现了巴宾斯基征。在90幅绘面的48幅中(占比53%)可观察到婴儿的脚底受到刺激。在所有Anilrea del Veroechio,Leonurdo da Vinei和Giorgione的画作中,都有两只脚出现巴宾斯基征的圣婴形象。画中是否出现巴宾斯基征和画家所处的时代并无关联。结论与幼年基督的绘画中出现巴宾斯基征有关的四个主要因素为:婴儿脚趾张开的生理现象;15世纪的画家们对于基督裸体的描绘;文艺复兴时期的画家对解剖结构精确的观察;以及一些菜的画派和弗菜芒画派画家对于准确细节的追求。而意大利文艺复兴画家们,无论是否为矫饰主义者,都顿向于理想化人体结构.所以不会在他们的画中再现巴宾基斯征。展开更多
基金supported by the funding from the Medical Research Council(MR/S006990/1)the Wellcome Trust(103191/A/13/Z)+3 种基金the Rosetrees Trust(M806)the UCL Neurogenetic Therapies Programme funded by The Sigrid Rausing Trustnamed inventor on patent GB2303495.2(patent applicant,UCL Business Ltd.,status pending)which describes and protects AAV-BDNF technology for treatment of CMT。
文摘Charcot-Marie-Tooth disease(CMT)is a hereditary peripheral neuropathy causing muscle weakness/wasting and sensory dysfunction predominantly in limb extremities.CMT patients display gait abnormalities,foot deformities,loss of sensation and decreased/absent deep tendon reflexes,with motor symptoms usually being more prominent than sensory.
基金This work was supported by grants from the National Natural Science Foundation of China(81570768 to Y.-C.W.,31900841 to Yan Li,91957205 and 31525012 to H.Y.,and 81471016 to J.J.)the Ministry of Science and Technology of China(2016YFA0500102 and 2016YFC1304905)CAS Key Laboratory of Nutrition,Metabolism and Food Safety(KLNMFS2019-01),and Chinese Academy of Sciences Interdisciplinary Innovation Team.
文摘Muscle regeneration after damage or during myopathies requires a fine cooperation between myoblast proliferation and myogenic differentiation.A growing body of evidence suggests that microRNAs play critical roles in myocyte proliferation and differentiation transcriptionally.However,the molecular mechanisms underlying the orchestration are not fully understood.Here,we showed that miR-130b is able to repress myoblast proliferation and promote myogenic differentiation via targeting Sp1 transcription factor.Importantly,overexpression of miR-130b is capable of improving the recovery of damaged muscle in a freeze injury model.Moreover,miR-130b expression is declined in the muscle of muscular dystrophy patients.Thus,these results indicated that miR-130b may play a role in skeletal muscle regeneration and myopathy progression.Together,our findings suggest that the miR-130b/Sp1 axis may serve as a potential therapeutic target for the treatment of patients with muscle damage or severe myopathies.
文摘目的系统调查文艺复兴时期的伟大画家如何在作品中呈现幼年耶稣的巴宾斯基征(Babinski sign)。设计观察性研究背景通过已发表的编目和谷歌。搜索大量公元1400-1550年期间弗菜芒画派(Flemish).莱茵面派(Rhenish)和意大利学院源两家绘制的幼年耶穌画作。研究样本302幅文艺复兴时期描绘幼年基督的画作(来自19位画家)。主要结局指标巴宾斯基征的定义为婴儿大脚趾张开幅度超过30度。还应注意来自足底的朝激。结果在302幅绘面中,有90幅绘画(占比约30%)中出现了明显向上张开的脚趾。在由Rogier van der Weyden,Hans Menling,Marin Schongouer和Mathias Grinewald创作的幼年基督的作品中,有超过60%出现了巴宾斯基征。甚至在3幅西中,罌儿的两只脚都出现了巴宾斯基征。在90幅绘面的48幅中(占比53%)可观察到婴儿的脚底受到刺激。在所有Anilrea del Veroechio,Leonurdo da Vinei和Giorgione的画作中,都有两只脚出现巴宾斯基征的圣婴形象。画中是否出现巴宾斯基征和画家所处的时代并无关联。结论与幼年基督的绘画中出现巴宾斯基征有关的四个主要因素为:婴儿脚趾张开的生理现象;15世纪的画家们对于基督裸体的描绘;文艺复兴时期的画家对解剖结构精确的观察;以及一些菜的画派和弗菜芒画派画家对于准确细节的追求。而意大利文艺复兴画家们,无论是否为矫饰主义者,都顿向于理想化人体结构.所以不会在他们的画中再现巴宾基斯征。
