Masaaki Komatsu

This study shows that reduced HOIL-1 activity increases Met1-ubiquitin chains, leading to solid protein aggregates and impaired autophagy in neurodegenerative diseases.

The paper shows that KEAP1 retention in p62 bodies and NRF2 activation cause liver damage when autophagy is impaired, and that blocking p62-KEAP1 interactions reduces this damage.

This study shows that loss of SPNS1 in the nervous system causes dysmyelination and white matter dysplasia in mice, suggesting a role for SPNS1 mutations in human congenital white matter disorders.