Differential effects of SORL1 deficiency on the endo-lysosomal network in human neurons and microglia

Philos Trans R Soc Lond B Biol Sci. 2024 Apr 8;379(1899):20220389. doi: 10.1098/rstb.2022.0389. Epub 2024 Feb 19.

Abstract

The endosomal gene SORL1 is a strong Alzheimer's disease (AD) risk gene that harbours loss-of-function variants causative for developing AD. The SORL1 protein SORL1/SORLA is an endosomal receptor that interacts with the multi-protein sorting complex retromer to traffic various cargo through the endo-lysosomal network (ELN). Impairments in endo-lysosomal trafficking are an early cellular symptom in AD and a novel therapeutic target. However, the cell types of the central nervous system are diverse and use the ELN differently. If this pathway is to be effectively therapeutically targeted, understanding how key molecules in the ELN function in various cell types and how manipulating them affects cell-type specific responses relative to AD is essential. Here, we discuss an example where deficiency of SORL1 expression in a human model leads to stress on early endosomes and recycling endosomes in neurons, but preferentially leads to stress on lysosomes in microglia. The differences observed in these organelles could relate to the unique roles of these cells in the brain as neurons are professional secretory cells and microglia are professional phagocytic cells. Experiments to untangle these differences are fundamental to advancing the understanding of cell biology in AD and elucidating important pathways for therapeutic development. Human-induced pluripotent stem cell models are a valuable platform for such experiments. This article is part of a discussion meeting issue 'Understanding the endo-lysosomal network in neurodegeneration'.

Keywords: Alzheimer's disease; SORL1; endosomal trafficking; microglia; neurons.

Publication types

  • Review

MeSH terms

  • Alzheimer Disease* / genetics
  • Brain / metabolism
  • Humans
  • LDL-Receptor Related Proteins / metabolism
  • Lysosomes / metabolism
  • Membrane Transport Proteins / genetics
  • Membrane Transport Proteins / metabolism
  • Microglia / metabolism
  • Neurons

Substances

  • SORL1 protein, human
  • LDL-Receptor Related Proteins
  • Membrane Transport Proteins