Emotion Evaluation and Social Inference Impairments in Huntington's Disease [0.03%]
亨廷顿病的情绪评价和社交推论障碍
April L Philpott,Sophie C Andrews,Mathew Staios et al.
April L Philpott et al.
Background: Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by motor, cognitive and neuropsychiatric symptoms. Recent research has established that individuals with HD display reduced di...
AUTEN-67 (Autophagy Enhancer-67) Hampers the Progression of Neurodegenerative Symptoms in a Drosophila model of Huntington's Disease [0.03%]
自噬增强剂AUTEN-67可阻碍亨廷顿舞蹈病果蝇模型中神经退行性症状的发展
Viktor Billes,Tibor Kovács,Bernadette Hotzi et al.
Viktor Billes et al.
Background: Autophagy, a lysosome-mediated self-degradation process of eukaryotic cells, serves as a main route for the elimination of cellular damage [1-3]. Such damages include aggregated, oxidized or misfolded proteins...
Neonatal Iron Supplementation Induces Striatal Atrophy in Female YAC128 Huntington's Disease Mice [0.03%]
新生铁补充诱导亨廷顿舞蹈病(YAC128)小鼠纹状体萎缩
Kiersten L Berggren,Zhen Lu,Julia A Fox et al.
Kiersten L Berggren et al.
Background: Dysregulation of iron homeostasis is implicated in the pathogenesis of Huntington's disease. We have previously shown that increased iron intake in R6/2 HD neonatal mice, but not adult R6/2 HD mice potentiates...
Partial Amelioration of Peripheral and Central Symptoms of Huntington's Disease via Modulation of Lipid Metabolism [0.03%]
通过调节脂质代谢部分改善亨廷顿舞蹈病的外周和中枢症状
Jane Y Chen,Conny Tran,Lin Hwang et al.
Jane Y Chen et al.
Background: Huntington's disease (HD) is a fatal, inherited neurodegenerative disorder characterized by uncontrollable dance-like movements, as well as cognitive deficits and mood changes. A feature of HD is a metabolic d...
Delayed Onset and Reduced Cognitive Deficits through Pre-Conditioning with 3-Nitropropionic Acid is Dependent on Sex and CAG Repeat Length in the R6/2 Mouse Model of Huntington's Disease [0.03%]
用3-硝基丙酸预处理可延缓发病并减少亨廷顿舞蹈病R6/2小鼠模型的认知缺陷：性别和CAG重复序列长度的影响不容忽视
Elizabeth A Skillings,A Jennifer Morton
Elizabeth A Skillings
Background: Impairments in energy metabolism are implicated in Huntington's disease (HD) pathogenesis. Reduced levels of the mitochondrial enzyme succinate dehydrogenase (SDH), the main element of complex II, are observed...
Lauren M Byrne,Edward J Wild
Lauren M Byrne
Cerebrospinal fluid (CSF) is enriched in brain-derived components and represents an accessible and appealing means of interrogating the CNS milieu to study neurodegenerative diseases and identify biomarkers to facilitate the development of ...
Does the Mutant CAG Expansion in Huntingtin mRNA Interfere with Exonucleolytic Cleavage of its First Exon? [0.03%]
亨廷顿mRNA中的突变CAG重复是否干扰其第一个外显子的降解？
Wanzhao Liu,Edith L Pfister,Lori A Kennington et al.
Wanzhao Liu et al.
Background: Silencing mutant huntingtin mRNA by RNA interference (RNAi) is a therapeutic strategy for Huntington's disease. RNAi induces specific endonucleolytic cleavage of the target HTT mRNA, followed by exonucleolytic...
Benton Judgment of Line Orientation (JoLO) Test: A Brief and Useful Measure for Assessing Visuospatial Abilities in Manifest, but not Premanifest, Huntington's Disease [0.03%]
苯顿线方向判断测验(HD患者视空间能力评估的简便工具)
Jody Corey-Bloom,Shea Gluhm,Andrew Herndon et al.
Jody Corey-Bloom et al.
Background: Visuospatial deficits have been described in Huntington's disease (HD); however, the extent of these deficits remains unclear. The Benton Judgment of Line Orientation (JoLO) Test, commonly used to assess visuo...
Participants at the Leiden Site of the REGISTRY Study: A Demographic Approach [0.03%]
REGISTRY研究利尔den登记处的参与者：一种人口学方法
Verena Baake,Ellen P Hart,Reineke Bos et al.
Verena Baake et al.
Background: REGISTRY is the largest European observational study of Huntington's disease (HD). The Leiden University Medical Center (LUMC) in The Netherlands is the largest recruiting site. ...
Brady P Culver,Josh DeClercq,Igor Dolgalev et al.
Brady P Culver et al.
Background: The Huntington's disease (HD) protein huntingtin (Htt) plays a role in multiple cellular pathways. Deregulation of one or more of these pathways by the mutant Htt protein has been suggested to contribute to th...