GSE137420
Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid  model of CLN3-Juvenile Neuronal Ceroid Lipofuscinosis
We developed an invitro model for Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) using isogenic CLN3 mutated human iPS cell lines and performed transcriptomic profiling of brain organoids derived from these lines to identify transcriptomic changes in the early developing brain model. Overall design: The study consists of mRNA-Seq (6 Control and 6 CLN3 mutant samples) performed in organoids differentiated for 55 days in vitro.