Blair R Leavitt,Leslie M Thompson
Blair R Leavitt
β-Defensin Genomic Copy Number Does Not Influence the Age of Onset in Huntington's Disease [0.03%]
β-防御素基因组拷贝数不影响亨廷顿舞蹈病的发病年龄
Angelica Vittori,Michael Orth,Raymund A C Roos et al.
Angelica Vittori et al.
Background: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by the abnormal expansion of a CAG triplet repeat tract in the huntingtin gene. While the length of this CAG expansion is th...
Decreasing Levels of the cdk5 Activators, p25 and p35, Reduces Excitotoxicity in Striatal Neurons [0.03%]
CDK5活化剂p25和p35水平的下降可减少纹状体神经元的兴奋毒性损伤
Kevin H J Park,Ge Lu,Jing Fan et al.
Kevin H J Park et al.
Huntington's disease (HD) is a progressive neurodegenerative disorder caused by an expanded CAG trinucleotide repeat sequence in the huntingtin gene. The resulting poly-glutamine expansion in the huntingtin protein imparts a novel toxic gai...
Direct evidence of progressive cardiac dysfunction in a transgenic mouse model of Huntington's disease [0.03%]
亨廷顿舞蹈病转基因小鼠心脏功能障碍的直接证据
Nigel I Wood,Stephen J Sawiak,Guido Buonincontri et al.
Nigel I Wood et al.
HD is a progressive genetic neurological disorder, characterized by motor as well as cognitive impairments. The gene carrying the mutation causing Huntington's disease (HD) is not brain specific, and there is increasing evidence for periphe...
Detection of Mutant Huntingtin Aggregation Conformers and Modulation of SDS-Soluble Fibrillar Oligomers by Small Molecules [0.03%]
小分子检测突变型亨廷顿蛋白聚集结构并调节其SDS可溶性纤维状寡聚体的能力
Emily Mitchell Sontag,Gregor P Lotz,Guocheng Yang et al.
Emily Mitchell Sontag et al.
The Huntington's disease (HD) mutation leads to a complex process of Huntingtin (Htt) aggregation into multimeric species that eventually form visible inclusions in cytoplasm, nuclei and neuronal processes. One hypothesis is that smaller, s...
Age-Dependent Resistance to Excitotoxicity in Htt CAG140 Mice and the Effect of Strain Background [0.03%]
HTT CAG140小鼠兴奋毒性抵抗的年龄依赖性及其背景基因型的影响
Melissa K Strong,Amber L Southwell,Jennifer M Yonan et al.
Melissa K Strong et al.
Mouse strain background can influence vulnerability to excitotoxic neuronal cell death and potentially modulate phenotypes in transgenic mouse models of human disease. Evidence supports a contribution of excitotoxicity to the selective deat...
Comparative Study
Journal of Huntington's disease. 2012;1(2):221-41. DOI:10.3233/JHD-129005 2012
Pizotifen Activates ERK and Provides Neuroprotection in vitro and in vivo in Models of Huntington's Disease [0.03%]
皮佐替酚激活ERK并在亨廷顿舞蹈病的体内和体外模型中发挥神经保护作用
Melissa R Sarantos,Theodora Papanikolaou,Lisa M Ellerby et al.
Melissa R Sarantos et al.
Background: Huntington's disease (HD) is a dominantly inherited neurodegenerative condition characterized by dysfunction in striatal and cortical neurons. There are currently no approved drugs known to slow the progressio...
Poly-glutamine expanded huntingtin dramatically alters the genome wide binding of HSF1 [0.03%]
多聚谷氨酰胺扩增的亨廷顿蛋白改变HSF1结合基因组的范围
Laura Riva,Martina Koeva,Ferah Yildirim et al.
Laura Riva et al.
In Huntington's disease (HD), polyglutamine expansions in the huntingtin (Htt) protein cause subtle changes in cellular functions that, over-time, lead to neurodegeneration and death. Studies have indicated that activation of the heat shock...
Genetic Deficiency of Complement Component 3 Does Not Alter Disease Progression in a Mouse Model of Huntington's Disease [0.03%]
补体成分C3遗传缺乏不影响亨廷顿舞蹈病小鼠模型的疾病进展
Paul B Larkin,Paul J Muchowski
Paul B Larkin
Several genes and proteins of the complement cascade are present at elevated levels in brains of patients with Huntington's disease (HD). The complement cascade is well characterized as an effector arm of the immune system, and in the brain...
Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice [0.03%]
猎тин틴脯氨酸富集区的缺失不影响小鼠中正常猎틴틴的功能
Michelle Neveklovska,Erin B D Clabough,Joan S Steffan et al.
Michelle Neveklovska et al.
The N-terminus of Huntingtin, the protein encoded by the Huntington's disease gene, contains a stretch of polyglutamine residues that is expanded in Huntington's disease. The polyglutamine stretch is flanked by two conserved protein domains...