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

Gemma Gomez-Giro, Jonathan Arias-Fuenzalida, Javier Jarazo, Dagmar Zeuschner, Muhammad Ali, Nina Possemis, Silvia Bolognin, Rashi Halder, Christian Jager, Willemijn F. E. Kuper, Peter M. van Hasselt, Holm Zaehres, Antonio del Sol, Herman van der Putten, Hans R. Scholer, Jens C. Schwamborn*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Original languageEnglish
Article number222
Number of pages19
JournalActa neuropathologica communications
Volume7
Issue number1
DOIs
Publication statusPublished - 30 Dec 2019

Keywords

  • JNCL
  • CLN3 disease
  • CRISPR/Cas9
  • Cerebral organoids
  • Neurodevelopment
  • Synapses
  • PLURIPOTENT STEM-CELLS
  • MOUSE MODEL
  • CATHEPSIN-D
  • PARKINSONS-DISEASE
  • LYSOSOMAL STORAGE
  • PROTEIN
  • CLN3
  • MUTATIONS
  • GENE
  • GENERATION

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