GRN−/− iPSC-derived cortical neurons recapitulate the pathological findings of both frontotemporal lobar degeneration and neuronal ceroidolipofuscinosis

GRN−/− iPSC-derived cortical neurons recapitulate the pathological findings of both frontotemporal lobar degeneration and neuronal ceroidolipofuscinosis

Heterozygous mutations in the gene coding for progranulin (GRN) cause frontotemporal lobar degeneration (FTLD) while homozygous mutations are linked to neuronal ceroidolipofuscinosis (NCL). While both FTLD/NCL pathological hallmarks were mostly investigated in heterozygous GRN+/− brain tissue or ind...

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Bibliographic Details
Main Authors: Patrizia Bossolasco, Sara Cimini, Emanuela Maderna, Donatella Bardelli, Laura Canafoglia, Tiziana Cavallaro, Martina Ricci, Vincenzo Silani, Gianluca Marucci, Giacomina Rossi
Format: Article
Language:English
Published: Elsevier 2022-12-01
Series:Neurobiology of Disease
Subjects:
Progranulin
Frontotemporal lobar degeneration
Neuronal ceroidolipofuscinosis
Induced pluripotent stem cells
Cortical neurons
Lysosomes
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996122002832

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http://www.sciencedirect.com/science/article/pii/S0969996122002832

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