Department of Pathology, University of California San Diego, USA.
Department of Neurology, Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK.
Neurobiol Dis. 2021 Apr;151:105273. doi: 10.1016/j.nbd.2021.105273. Epub 2021 Jan 19.
Pathological hyperphosphorylated tau is a key feature of Alzheimer's disease (AD) and Frontotemporal dementia (FTD). Using transgenic mice overexpressing human non-mutated tau (htau mice), we assessed the contribution of tau to peripheral and central neurodegeneration. Indices of peripheral small and large fiber neuropathy and learning and memory performances were assessed at 3 and 6 months of age. Overexpression of human tau is associated with peripheral neuropathy at 6 months of age. Our study also provides evidence that non-mutated tau hyperphosphorylation plays a critical role in memory deficits. In addition, htau mice had reduced stromal corneal nerve length with preservation of sub-basal corneal nerves, consistent with a somatofugal degeneration. Corneal nerve degeneration occurred prior to any cognitive deficits and peripheral neuropathy. Stromal corneal nerve loss was observed in patients with FTD but not AD. Corneal confocal microscopy may be used to identify early neurodegeneration and differentiate FTD from AD.
病理性过度磷酸化的 tau 蛋白是阿尔茨海默病 (AD) 和额颞叶痴呆 (FTD) 的一个关键特征。我们使用过度表达人源非突变 tau 蛋白的转基因小鼠 (htau 小鼠) ,评估 tau 蛋白对周围和中枢神经退行性变的影响。在 3 个月和 6 个月大时,评估周围小纤维和大纤维神经病以及学习和记忆表现的指标。在 6 个月大时,人源 tau 蛋白的过度表达与周围神经病有关。我们的研究还提供了证据,表明非突变 tau 蛋白过度磷酸化在记忆缺陷中起着关键作用。此外,htau 小鼠的基质角膜神经长度减少,而基底下角膜神经保持不变,这与躯体远心性退行性变一致。角膜神经退行性变发生在任何认知缺陷和周围神经病之前。在 FTD 患者中观察到基质角膜神经丢失,但在 AD 患者中没有。角膜共聚焦显微镜检查可能用于识别早期神经退行性变,并将 FTD 与 AD 区分开来。