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

doi:10.1186/s40478-019-0871-7

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 journal › Article › Academic › peer-review

Original language	English
Article number	222
Number of pages	19
Journal	Acta neuropathologica communications
Volume	7
Issue number	1
DOIs	https://doi.org/10.1186/s40478-019-0871-7
Publication status	Published - 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

Access to Document

10.1186/s40478-019-0871-7Licence: CC BY

Cite this

Gomez-Giro, G., Arias-Fuenzalida, J., Jarazo, J., Zeuschner, D., Ali, M., Possemis, N., Bolognin, S., Halder, R., Jager, C., Kuper, W. F. E., van Hasselt, P. M., Zaehres, H., del Sol, A., van der Putten, H., Scholer, H. R., & Schwamborn, J. C. (2019). Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis. Acta neuropathologica communications, 7(1), [222]. https://doi.org/10.1186/s40478-019-0871-7

Gomez-Giro, Gemma ; Arias-Fuenzalida, Jonathan ; Jarazo, Javier ; Zeuschner, Dagmar ; Ali, Muhammad ; Possemis, Nina ; Bolognin, Silvia ; Halder, Rashi ; Jager, Christian ; Kuper, Willemijn F. E. ; van Hasselt, Peter M. ; Zaehres, Holm ; del Sol, Antonio ; van der Putten, Herman ; Scholer, Hans R. ; Schwamborn, Jens C. / Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis. In: Acta neuropathologica communications. 2019 ; Vol. 7, No. 1.

@article{7db1668ee9034f1fbac20726e8346763,

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

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",

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

year = "2019",

month = dec,

day = "30",

doi = "10.1186/s40478-019-0871-7",

language = "English",

volume = "7",

journal = "Acta neuropathologica communications",

issn = "2051-5960",

publisher = "BioMed Central Ltd",

number = "1",

}

Gomez-Giro, G, Arias-Fuenzalida, J, Jarazo, J, Zeuschner, D, Ali, M, Possemis, N, Bolognin, S, Halder, R, Jager, C, Kuper, WFE, van Hasselt, PM, Zaehres, H, del Sol, A, van der Putten, H, Scholer, HR & Schwamborn, JC 2019, 'Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis', Acta neuropathologica communications, vol. 7, no. 1, 222. https://doi.org/10.1186/s40478-019-0871-7

Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis. / Gomez-Giro, Gemma; Arias-Fuenzalida, Jonathan; Jarazo, Javier; Zeuschner, Dagmar; Ali, Muhammad; Possemis, Nina; Bolognin, Silvia; Halder, Rashi; Jager, Christian; Kuper, Willemijn F. E.; van Hasselt, Peter M.; Zaehres, Holm; del Sol, Antonio; van der Putten, Herman; Scholer, Hans R.; Schwamborn, Jens C.

In: Acta neuropathologica communications, Vol. 7, No. 1, 222, 30.12.2019.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis

AU - Gomez-Giro, Gemma

AU - Arias-Fuenzalida, Jonathan

AU - Jarazo, Javier

AU - Zeuschner, Dagmar

AU - Ali, Muhammad

AU - Possemis, Nina

AU - Bolognin, Silvia

AU - Halder, Rashi

AU - Jager, Christian

AU - Kuper, Willemijn F. E.

AU - van Hasselt, Peter M.

AU - Zaehres, Holm

AU - del Sol, Antonio

AU - van der Putten, Herman

AU - Scholer, Hans R.

AU - Schwamborn, Jens C.

PY - 2019/12/30

Y1 - 2019/12/30

KW - JNCL

KW - CLN3 disease

KW - CRISPR/Cas9

KW - Cerebral organoids

KW - Neurodevelopment

KW - Synapses

KW - PLURIPOTENT STEM-CELLS

KW - MOUSE MODEL

KW - CATHEPSIN-D

KW - PARKINSONS-DISEASE

KW - LYSOSOMAL STORAGE

KW - PROTEIN

KW - CLN3

KW - MUTATIONS

KW - GENE

KW - GENERATION

U2 - 10.1186/s40478-019-0871-7

DO - 10.1186/s40478-019-0871-7

M3 - Article

C2 - 31888773

VL - 7

JO - Acta neuropathologica communications

JF - Acta neuropathologica communications

SN - 2051-5960

IS - 1

M1 - 222

ER -