Jeh-Ping Liu,Scott O Zeitlin
Jeh-Ping Liu
Huntingtin (HTT) is an essential protein during early embryogenesis and the development of the central nervous system (CNS). Conditional knock-out of mouse Huntingtin (Htt) expression in the CNS beginning during neural development, as well ...
David G Anderson,Ruth H Walker,Myles Connor et al.
David G Anderson et al.
Background: Huntington Disease-like 2 (HDL2) is a neurodegenerative disorder similar to Huntington Disease (HD) in its clinical phenotype, genetic characteristics, neuropathology and longitudinal progression. Proposed spe...
Early Huntington's Disease: Impulse Control Deficits but Correct Judgment Regarding Risky Situations [0.03%]
早期亨廷顿舞蹈病：冲动控制缺陷但对高风险情境的判断能力完好
Victor Galvez,Juan Fernandez-Ruiz,Leo Bayliss et al.
Victor Galvez et al.
Background: Huntington's disease (HD) patients show alterations in decision making tasks. However, it is still uncertain if these deficits are due to poor judgment regarding risky situations, or to impulse control deficit...
N-Terminal Fragments of Huntingtin Longer than Residue 170 form Visible Aggregates Independently to Polyglutamine Expansion [0.03%]
亨廷顿蛋白第170位残基以上的N端片段能独立于多聚谷氨酰胺重复形成可见聚集物
Moore Z Chen,Sue-Ann Mok,Angelique R Ormsby et al.
Moore Z Chen et al.
Background: A hallmark of Huntington's disease is the progressive aggregation of full length and N-terminal fragments of polyglutamine (polyQ)-expanded Huntingtin (Htt) into intracellular inclusions. The production of N-t...
The Effect of Music Therapy in Patients with Huntington's Disease: A Randomized Controlled Trial [0.03%]
赫恩特顿病患者音乐疗法效果的随机对照试验
Monique C H van Bruggen-Rufi,Annemieke C Vink,Ron Wolterbeek et al.
Monique C H van Bruggen-Rufi et al.
Background: Music therapy may have beneficial effects on improving communication and expressive skills in patients with Huntington's disease (HD). Most studies are, however, small observational studies and methodologicall...
Randomized Controlled Trial
Journal of Huntington's disease. 2017;6(1):63-72. DOI:10.3233/JHD-160229 2017
Generation and Characterization of Knock-in Mouse Models Expressing Versions of Huntingtin with Either an N17 or a Combined PolyQ and Proline-Rich Region Deletion [0.03%]
表达亨廷顿蛋白N17或聚谷氨酰胺及脯氨酸丰富区域缺失版本的敲入小鼠模型的建立与表征
Emily A André,Elise M Braatz,Jeh-Ping Liu et al.
Emily A André et al.
Background: The polyglutamine (polyQ) stretch of the Huntingtin protein (HTT) in mammals is flanked by a highly conserved 17 amino acid N-terminal domain (N17), and a proline-rich region (PRR). The PRR is a binding site f...
Stewarding Hope: The Evolving Landscape of Huntington's Disease Science Communications [0.03%]
守望希望：亨廷顿舞蹈症科学传播的演变景观
George J Yohrling,Louise A Vetter
George J Yohrling
For individuals faced with Huntington's disease (HD), there is no lack of access to information about the newest HD research topics and care trends thanks to social media and online news feeds. Unfortunately, making sense of this volume of ...
Tuyana B Malankhanova,Anastasia A Malakhova,Sergey P Medvedev et al.
Tuyana B Malankhanova et al.
The development of new revolutionary technologies for directed gene editing has made it possible to thoroughly model and study NgAgo human diseases at the cellular and molecular levels. Gene editing tools like ZFN, TALEN, CRISPR-based syste...
Survey of the Huntington's Disease Patient and Caregiver Community Reveals Most Impactful Symptoms and Treatment Needs [0.03%]
亨廷顿病患者及看护者社区调研揭示最具有影响性的症状和治疗需求
Jennifer A Simpson,Debra Lovecky,Jane Kogan et al.
Jennifer A Simpson et al.
Background: In preparation for a meeting with the U.S. Food and Drug Administration (FDA) on Patient-Focused Drug Development in Huntington's disease, the Huntington's Disease Society of America (HDSA) created and distrib...
LBH589, A Hydroxamic Acid-Derived HDAC Inhibitor, is Neuroprotective in Mouse Models of Huntington's Disease [0.03%]
HDAC抑制剂LBH589对亨廷顿舞蹈病小鼠模型的神经保护作用
Vanita Chopra,Luisa Quinti,Prarthana Khanna et al.
Vanita Chopra et al.
Background: Modulation of gene transcription by HDAC inhibitors has been shown repeatedly to be neuroprotective in cellular, invertebrate, and rodent models of Huntington's disease (HD). It has been difficult to translate...