中国神经再生研究(英文版) ›› 2025, Vol. 20 ›› Issue (11): 3217-3218.doi: 10.4103/NRR.NRR-D-24-00639

• 观点:退行性病与再生 • 上一篇    下一篇

从终点开始:突触前末端作为额颞叶痴呆和肌萎缩侧索硬化症的病理机制中心

  

  • 出版日期:2025-11-15 发布日期:2025-02-23

Beginning from the end: the presynaptic terminal as a pathomechanism hub in frontotemporal dementia and amyotrophic lateral sclerosis

Laura Huggon, Emma L. Clayton*   

  1. UK Dementia Research Institute at King’s College London, London, UK (Huggon L, Clayton EL) Department of Basic and Clinical Neuroscience, Institute of Psychiatry Psychology & Neuroscience, King’s College London, London, UK (Huggon L, Clayton EL)
  • Online:2025-11-15 Published:2025-02-23
  • Contact: Emma L. Clayton, PhD, emma.clayton@kcl.ac.uk.
  • Supported by:
    ELC is funded by a senior research fellowship from Alzheimer’s Research UK (ARUK-SRF2022A-005). LH is funded by a PhD studentship from Alzheimer’s Society (571). This work is supported by the UK Dementia Research Institute through UK DRI Ltd., principally funded by the UK Medical Research Council. No conflicts of interest exist between UK DRI Ltd. and publication of this article.

摘要: https://orcid.org/0000-0002-0872-4762 (Laura Huggon) https://orcid.org/0000-0003-0937-2874 (Emma L. Clayton)

Abstract: Frontotemporal dementia and amyotrophic lateral sclerosis: Frontotemporal dementia ( F T D ) a n d a m y o t r o p h i c l a t e r a l s c l e r o s i s (ALS) are neurodegenerative diseases with significant overlapping attributes. While these neurodegenerative diseases affect different neuronal populations (with FTD affecting neurons of the frontal and temporal lobes, and ALS affecting upper and lower motor neurons), these two diseases are complexly intertwined. FTD and ALS exist on a disease spectrum, with shared genetic causes, clinical presentations, and pathologies. The continuum of the genetic disease spectrum runs from those genetic mutations that cause ALS (SOD-1) through those that can cause ALS/FTD (TDP-43, FUS, CHCHD10, C9ORF72, TBK-1, UBQLN2, and CHMP2B) to those that cause FTD (MAPT and PGRN) (reviewed in Abramzon et al., 2020). Models of these genetic causes of disease have been instrumental in advancing our understanding of disease mechanisms.