Molecular Neuropathobiology Laboratory

Understanding the molecular pathogenesis of neurodegenerative diseases: towards the development of effective therapeutic agents for the treatment of ALS and other neurodegenerative diseases

Amyotrophic lateral sclerosis (ALS) is a heterogeneous group of incurable motor neuron diseases (MNDs) characterized by a selective loss of upper and lower motor neurons in the brain and spinal cord.

In 2001, we have identified ALS2 as a causative gene for a recessive type of familial ALS. The ALS2 gene encodes ALS2 (or alsin) that is implicated in axonal outgrowth, cytoprotection from oxidative stress, and proteostasis by regulating the autophagy-endlysoosomal system. However, our understanding of the intricate autophagy-endolysosomal system and its functional linking to other physiological systems in the CNS is still immature.

In our lab, to uncover the molecular mechanisms of a selective neurodegeneration, we are currently focusing on the autophagy-lysosomal and endocytic degradation systems, and implication of their dysfunction to the pathogenesis of ALS/MNDs. Ultimately, through these studies, we aim to develop effective therapeutic agents for the treatment of ALS/MNDs and other neurodegenerative diseases.


  • Updated on May 6th, 2021
  • We are looking for enthusiastic and highly motivated graduate and undergraduate students who want to join our lab.
  • New review publication: Klionsky, D. J. et al. Autophagy 17 (1), 1-382. (2021.3.1)
  • New publication: Nozaki, M. et al. eNeurologicalSci 22, 100301. (2020.12.8)
  • New publication: Oka, A. et al. EBioMedicine 57, 102810. (2020.6.21)
  • New publication: Hayashi, H. et al. PLoS ONE 15 (6), e0234180. (2020.6.8)
  • New publication: Otomo, A. et al. Sci. Rep. 10 (1), 6716 (2020.4.21)