Slow Elizabeth J, Graham Rona K, Hayden Michael R
Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, Department of Medical Genetics, University of British Columbia, Vancouver V5Z 4H4, BC, Canada.
Trends Genet. 2006 Aug;22(8):408-11. doi: 10.1016/j.tig.2006.05.008. Epub 2006 Jun 27.
Insoluble aggregated proteins in Alzheimer disease and Huntington disease might not be pathogenic. Human studies have poor correlations between aggregates and clinical disease or pathology in these disorders, whereas mouse models have demonstrated that neuronal loss can occur in the absence of detectable aggregates. Furthermore, aggregates can exist in the absence of disease pathology in mice or symptoms in humans. Recent research suggests that soluble protein fragments, not insoluble aggregated proteins, are the toxic species in these disorders.
阿尔茨海默病和亨廷顿病中不溶性聚集蛋白可能并无致病性。人体研究表明，在这些疾病中，聚集蛋白与临床疾病或病理学之间的相关性较弱，而小鼠模型显示，在未检测到聚集蛋白的情况下也可发生神经元丢失。此外，在小鼠中，无疾病病理学表现时可存在聚集蛋白，在人类中，无疾病症状时也可存在聚集蛋白。最近的研究表明，在这些疾病中，有毒有毒有毒的毒性物质是可溶性蛋白片段，而非不溶性聚集蛋白。
