中国神经再生研究(英文版) ›› 2025, Vol. 20 ›› Issue (1): 195-196.doi: 10.4103/NRR.NRR-D-23-01753

• 观点:退行性病与再生 • 上一篇    下一篇

神经退行性疾病细胞器完整性的分区调控:从果蝇运动神经元的经验教训

  

  • 出版日期:2025-01-15 发布日期:2025-01-15

Compartmentalized regulation of organelle integrity in neurodegenerative diseases: lessons from the Drosophila motor neuron

Hyun Sung*   

  1. Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Medical Research Institute, and Department of Pharmacology, Pusan National University School of Medicine, Yangsan, Republic of Korea
  • Online:2025-01-15 Published:2025-01-15
  • Contact: Hyun Sung, PhD, hsung9@jhmi.edu or hsung@pusan.ac.kr.
  • Supported by:
    This work was supported by the Merkin PNNR Center (23-DF/C2/261) (to HS).

摘要: https://orcid.org/0000-0003-3451-1442 (Hyun Sung)

Abstract: Neurons are highly polarized, morphologically asymmetric, and functionally compartmentalized cells that contain long axons extending from the cell body. For this reason, their maintenance relies on spatiotemporal regulation of organelle distribution between the somatodendritic and axonal domains. Although some organelles, such as mitochondria and smooth endoplasmic reticulum, are widely distributed throughout the neuron, others are segregated to either the somatodendritic or axonal compartment. For example, Golgi outposts and acidified lysosomes are predominantly present in the somatodendritic domain and rarely distributed along the axon, whereas newly formed autophagosomes and synaptic vesicles are mainly distributed in the distal axon (Britt et al., 2016). To establish these polarized features, neurons regulate the axonal transport of organelles to maintain their distribution, integrity, and dynamics. Thus, it is not surprising that neurons in neurodegenerative diseases display early and diverse defects in organelle structure, distribution, and dynamics.