Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis

Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis

Abstract The juvenile form of neuronal ceroid Lipofuscinosis (JNCL) is the most common form within this group of rare lysosomal storage disorders, causing pediatric neurodegeneration. The genetic disorder, which is caused by recessive mutations affecting the CLN3 gene, features progressive vision lo...

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Bibliographic Details
Main Authors: Gemma Gomez-Giro, Jonathan Arias-Fuenzalida, Javier Jarazo, Dagmar Zeuschner, Muhammad Ali, Nina Possemis, Silvia Bolognin, Rashi Halder, Christian Jäger, Willemijn F. E. Kuper, Peter M. van Hasselt, Holm Zaehres, Antonio del Sol, Herman van der Putten, Hans R. Schöler, Jens C. Schwamborn
Format: Article
Language:English
Published: BMC 2019-12-01
Series:Acta Neuropathologica Communications
Subjects:
JNCL
CLN3 disease
CRISPR/Cas9
Cerebral organoids
Neurodevelopment
Synapses
Online Access:https://doi.org/10.1186/s40478-019-0871-7

Internet

https://doi.org/10.1186/s40478-019-0871-7

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