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Article

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

Stem cell reports - 14 Mar 2023

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

Abstract excerpt

Loss of function (LoF) of TAR-DNA binding protein 43 (TDP-43) and mis-localization, together with TDP-43-positive and hyperphosphorylated inclusions, are found in post-mortem tissue of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) patients, including those carrying LoF variants in the progranulin gene (GRN). Modeling TDP-43 pathology has been challenging in vivo and in vitro. We present a...

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Identifiers

PMID: 36827976
DOI: 10.1016/j.stemcr.2023.01.012
PMCID: PMC10031303

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