β-Blocker effects in huntington's disease: A caution on clinical interpretation [0.03%]
亨廷顿舞蹈病的β阻滞剂效应:临床解释需谨慎
N Ahmad Aziz,Daniel Claassen,Åsa Petersén et al.
N Ahmad Aziz et al.
A recent retrospective analysis of Enroll HD data suggesting β-blockers slow Huntington's disease progression has triggered patient demand but requires caution. The findings rely solely on small observational subsets and are vulnerable to ...
Insights into neurodevelopmental features of Huntington's disease from stem cell-derived models including organoids [0.03%]
基于干细胞(包括类器官)模型的亨廷顿舞蹈病神经发育特征的研究进展
Mariana Sierra,Rachael Powers,Nikolas Grotewold et al.
Mariana Sierra et al.
Two- and three-dimensional (2D and 3D) cell models derived from human stem cells have shed light on a wide range of molecular and cellular features of Huntington's disease (HD). Here we review the use of human stem cell-derived models to ex...
Handgrip strength and the Index of pulmonary dysfunction: Practical screening tools for cough dysfunction in huntington's disease [0.03%]
握手力和肺功能指数:亨廷顿舞蹈病咳嗽功能障碍的实用筛查工具
Romana Konvalinkova,Martin Srp,Kristyna Doleckova et al.
Romana Konvalinkova et al.
BackgroundAs Huntington's disease (HD) progresses, it impairs airway protection, increasing the risk of aspiration pneumonia-the leading cause of death in HD. Although voluntary peak cough flow (vPCF) assesses cough effectiveness, its clini...
Effects of computerized cognitive training on functional activity and connectivity in Huntington's disease: A pilot study [0.03%]
计算机认知训练对亨廷顿病患者功能活动和脑网络连接的影响:一项初步研究
Katharine Huynh,Nellie Georgiou-Karistianis,Amit Lampit et al.
Katharine Huynh et al.
BackgroundComputerized cognitive training (CCT) has been found to improve cognition by altering functional activity and functional connectivity of brain networks in people with and without cognitive impairment. The effects of CCT on functio...
Mena Farag,Sarah J Tabrizi,Edward J Wild
Mena Farag
In this edition of the Huntington's Disease Clinical Trials Update, we expand on the launch of the phase II/III clinical trial of SKY-0515 from Skyhawk Therapeutics and the phase I/II clinical trial of SPK-10001 from Spark Therapeutics. We ...
Huntington's as a developmental disorder: Molecular and neuropathologic considerations [0.03%]
亨廷顿病的发育特性:分子和神经病理学考虑因素
Mallory R Shin,Marco M Hefti
Mallory R Shin
Huntington's disease (HD) is classically characterized as a late-onset neurodegenerative disorder of adulthood caused by CAG expansion in the HTT gene. However, mounting evidence from both human and experimental studies suggests that both w...
Early life functional advantage coupled with accelerated aging: The case for antagonistic pleiotropy in Huntington's disease [0.03%]
早年功能优势与加速衰老相结合:亨廷顿舞蹈病中对抗性多效性的案例
Jordan L Schultz,Peg C Nopoulos
Jordan L Schultz
Recent findings suggest that neurodevelopment plays a critical role in Huntington's Disease (HD) pathogenesis. This review integrates data from human studies of children and young adults at risk for HD (the Kids-HD study) with the theory of...
Emotion recognition in people with Huntington's disease: A comprehensive systematic review [0.03%]
亨廷顿病患者的情绪识别:全面的系统综述
Nicolò Zarotti,Alice Storey,Sarah Lloyd et al.
Nicolò Zarotti et al.
BackgroundDeficits of emotion recognition have received increasing attention in people with Huntington's disease (HD) in the three decades since the discovery of the HD gene. However, the characterisation of such deficits across different d...
Huntingtin knockdown dysregulates autophagic degradation of Apolipoprotein E [0.03%]
亨廷顿蛋白敲低干扰载脂蛋白E的自噬降解作用
Gianna M Fote,Nicolette R McClure,Robert M Bragg et al.
Gianna M Fote et al.
BackgroundThe HTT protein, mutated in Huntington's disease, is expressed throughout the body, and loss of HTT function as an autophagic scaffold may affect tissues and cellular processes. These processes include lipid metabolism potentially...
Morphological and functional phenotyping of skeletal muscle and bone in the zQ175 knock-in mouse model of Huntington's disease [0.03%]
亨廷顿舞蹈病zQ175基因敲入小鼠骨骼肌和骨的形态学及功能表型研究
Behnaz Nateghi,Mohamed Lala Bouali,Zineb Bouredji et al.
Behnaz Nateghi et al.
Background: Huntington's disease (HD) is a progressive neurodegenerative disorder primarily affecting the central nervous system (CNS). However, emerging evidence suggests that peripheral tissues, including skeletal muscle and bone, also un...