Neuronal intranuclear inclusion disease(NIID) is a rare autosomal dominant neurodegenerative disorder defined by the presence of eosinophilic intranuclear inclusions across both central and peripheral components of th...Neuronal intranuclear inclusion disease(NIID) is a rare autosomal dominant neurodegenerative disorder defined by the presence of eosinophilic intranuclear inclusions across both central and peripheral components of the nervous system,as well as multiple visceral organs,resulting in pronounced clinical heterogeneity.Following the discovery of pathogenic GGC repeat expansions in the NOTCH2NLC gene as the underlying genetic driver,a diverse array of experimental platforms has been established to probe NIID pathogenesis,including adenoassociated virus-mediated expression systems,transgenic animal models,and patient-derived cellular systems such as brain organoids.Collectively,these models recapitulate key histopathological and behavioral phenotypes observed in NIID and have elucidated multiple molecular and cellular pathways implicated in disease progression.This review systematically examines the current landscape of NIID model systems,highlighting their respective contributions to understanding disease pathogenesis,evaluating their experimental limitations,and identifying avenues for future refinement.Such integrative analysis is critical for advancing the development of more faithful disease models and facilitating the identification of therapeutic targets for NIID.展开更多
针对武器装备供应商选择问题,提出了一种基于灰色群组(grey group clustering,GGC)和改进标准间冲突性相关性(improved criteria importance through intercriteria correlation,ICRITIC)组合赋权的扩展多属性妥协解(VIKOR)决策方法。...针对武器装备供应商选择问题,提出了一种基于灰色群组(grey group clustering,GGC)和改进标准间冲突性相关性(improved criteria importance through intercriteria correlation,ICRITIC)组合赋权的扩展多属性妥协解(VIKOR)决策方法。采用考虑决策专家意见差异程度的群层次分析法对供应商初选指标体系进行降维遴选,构建武器装备供应商优选指标体系;结合GGC和ICRITIC组合赋权方法确定主客观权重,并设计权偏好系数进行加法组合赋权;基于VIKOR决策方法集结备选供应商折衷排序值。针对不同折衷系数、排序方法和赋权方法从决策灵活性和稳定性两个维度进行对比分析,验证所提方法的有效性。展开更多
In the originally published version of this paper,the images for GR5-YC+RGG1-YN and GR5-YC+GGC2-YN in Figure 4B were inadvertent duplicates due to an oversight during the figure assembly process.A corrected Figure 4B,...In the originally published version of this paper,the images for GR5-YC+RGG1-YN and GR5-YC+GGC2-YN in Figure 4B were inadvertent duplicates due to an oversight during the figure assembly process.A corrected Figure 4B,in which the panel for GR5-YC+RGG1-YN has been replaced with the correct image,is presented below,and the original article has been updated online.展开更多
基金supported by the National Key R&D Program of China (2021YFA0805200 to H.J.)National Natural Science Foundation of China (82394421 to B.S.T., 32071037 to Q.L., 82171843 to Y.C.P.)Natural Science Foundation of Hunan Province (2023JJ10097to Q.L., 2025JJ20079 to Y.C.P.)。
文摘Neuronal intranuclear inclusion disease(NIID) is a rare autosomal dominant neurodegenerative disorder defined by the presence of eosinophilic intranuclear inclusions across both central and peripheral components of the nervous system,as well as multiple visceral organs,resulting in pronounced clinical heterogeneity.Following the discovery of pathogenic GGC repeat expansions in the NOTCH2NLC gene as the underlying genetic driver,a diverse array of experimental platforms has been established to probe NIID pathogenesis,including adenoassociated virus-mediated expression systems,transgenic animal models,and patient-derived cellular systems such as brain organoids.Collectively,these models recapitulate key histopathological and behavioral phenotypes observed in NIID and have elucidated multiple molecular and cellular pathways implicated in disease progression.This review systematically examines the current landscape of NIID model systems,highlighting their respective contributions to understanding disease pathogenesis,evaluating their experimental limitations,and identifying avenues for future refinement.Such integrative analysis is critical for advancing the development of more faithful disease models and facilitating the identification of therapeutic targets for NIID.
文摘针对武器装备供应商选择问题,提出了一种基于灰色群组(grey group clustering,GGC)和改进标准间冲突性相关性(improved criteria importance through intercriteria correlation,ICRITIC)组合赋权的扩展多属性妥协解(VIKOR)决策方法。采用考虑决策专家意见差异程度的群层次分析法对供应商初选指标体系进行降维遴选,构建武器装备供应商优选指标体系;结合GGC和ICRITIC组合赋权方法确定主客观权重,并设计权偏好系数进行加法组合赋权;基于VIKOR决策方法集结备选供应商折衷排序值。针对不同折衷系数、排序方法和赋权方法从决策灵活性和稳定性两个维度进行对比分析,验证所提方法的有效性。
文摘In the originally published version of this paper,the images for GR5-YC+RGG1-YN and GR5-YC+GGC2-YN in Figure 4B were inadvertent duplicates due to an oversight during the figure assembly process.A corrected Figure 4B,in which the panel for GR5-YC+RGG1-YN has been replaced with the correct image,is presented below,and the original article has been updated online.
