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

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 loss, cogni...

Descripción completa

Detalles Bibliográficos
Autores principales: Gomez-Giro, Gemma, Arias-Fuenzalida, Jonathan, Jarazo, Javier, Zeuschner, Dagmar, Ali, Muhammad, Possemis, Nina, Bolognin, Silvia, Halder, Rashi, Jäger, Christian, Kuper, Willemijn F. E., van Hasselt, Peter M., Zaehres, Holm, del Sol, Antonio, van der Putten, Herman, Schöler, Hans R., Schwamborn, Jens C.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6937812/
https://www.ncbi.nlm.nih.gov/pubmed/31888773
http://dx.doi.org/10.1186/s40478-019-0871-7

Ejemplares similares

Ejemplares similares