Regulation of mitophagy by the NSL complex underlies genetic risk for Parkinson's disease at 16q11.2 and MAPT H1 loci

Brain. 2022 Dec 19;145(12):4349-4367. doi: 10.1093/brain/awac325.

Abstract

Parkinson's disease is a common incurable neurodegenerative disease. The identification of genetic variants via genome-wide association studies has considerably advanced our understanding of the Parkinson's disease genetic risk. Understanding the functional significance of the risk loci is now a critical step towards translating these genetic advances into an enhanced biological understanding of the disease. Impaired mitophagy is a key causative pathway in familial Parkinson's disease, but its relevance to idiopathic Parkinson's disease is unclear. We used a mitophagy screening assay to evaluate the functional significance of risk genes identified through genome-wide association studies. We identified two new regulators of PINK1-dependent mitophagy initiation, KAT8 and KANSL1, previously shown to modulate lysine acetylation. These findings suggest PINK1-mitophagy is a contributing factor to idiopathic Parkinson's disease. KANSL1 is located on chromosome 17q21 where the risk associated gene has long been considered to be MAPT. While our data do not exclude a possible association between the MAPT gene and Parkinson's disease, they provide strong evidence that KANSL1 plays a crucial role in the disease. Finally, these results enrich our understanding of physiological events regulating mitophagy and establish a novel pathway for drug targeting in neurodegeneration.

Keywords: GWAS; KANSL1; KAT8; Parkinson’s disease; mitophagy.

Publication types

  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Genome-Wide Association Study
  • Humans
  • Mitophagy* / physiology
  • Neurodegenerative Diseases
  • Parkinson Disease* / metabolism
  • Protein Kinases / genetics
  • tau Proteins / genetics

Substances

  • MAPT protein, human
  • Protein Kinases
  • tau Proteins
  • PTEN-induced putative kinase
  • KAT8 protein, human
  • NSL1 protein, human