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Granulin loss of function in human mature brain organoids implicates astrocytes in TDP-43 pathology

Authors
  • de Majo, Martina
  • Koontz, Mark
  • Marsan, Elise
  • Salinas, Nir
  • Ramsey, Arren
  • Kuo, Yien-Ming
  • Seo, Kyounghee
  • Li, Huinan
  • Dräger, Nina
  • Leng, Kun
  • Gonzales, Santiago L.
  • Kurnellas, Michael
  • Miyaoka, Yuichiro
  • Klim, Joseph R.
  • Kampmann, Martin
  • Ward, Michael E.
  • Huang, Eric J.
  • Ullian, Erik M.
Type
Published Article
Journal
Stem Cell Reports
Publisher
Elsevier
Publication Date
Feb 23, 2023
Volume
18
Issue
3
Pages
706–719
Identifiers
DOI: 10.1016/j.stemcr.2023.01.012
PMID: 36827976
PMCID: PMC10031303
Source
PubMed Central
Keywords
Disciplines
  • Article
License
Unknown

Abstract

In this article, de Majo et al. present a novel 3D iPSC-derived model to study neurodegenerative disorders such as ALS and FTD. When devoid of GRN expression, these cultures present features of ALS- and FTD-associated pathology hardly ever observed in vitro . These phenotypes are shown to be primarily driven by diseased astrocytes and can be rescued by pro-granulin supplementation.

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