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@ARTICLE{Buchner:258922,
author = {Buchner, Felix and Dokuzluoglu, Zeynep and Grass, Tobias
and Rodriguez-Muela, Natalia},
title = {{S}pinal {C}ord {O}rganoids to {S}tudy {M}otor {N}euron
{D}evelopment and {D}isease.},
journal = {Life},
volume = {13},
number = {6},
issn = {2075-1729},
address = {Basel},
publisher = {MDPI},
reportid = {DZNE-2023-00694},
pages = {1254},
year = {2023},
abstract = {Motor neuron diseases (MNDs) are a heterogeneous group of
disorders that affect the cranial and/or spinal motor
neurons (spMNs), spinal sensory neurons and the muscular
system. Although they have been investigated for decades, we
still lack a comprehensive understanding of the underlying
molecular mechanisms; and therefore, efficacious therapies
are scarce. Model organisms and relatively simple
two-dimensional cell culture systems have been instrumental
in our current knowledge of neuromuscular disease pathology;
however, in the recent years, human 3D in vitro models have
transformed the disease-modeling landscape. While cerebral
organoids have been pursued the most, interest in spinal
cord organoids (SCOs) is now also increasing. Pluripotent
stem cell (PSC)-based protocols to generate SpC-like
structures, sometimes including the adjacent mesoderm and
derived skeletal muscle, are constantly being refined and
applied to study early human neuromuscular development and
disease. In this review, we outline the evolution of human
PSC-derived models for generating spMN and recapitulating
SpC development. We also discuss how these models have been
applied to exploring the basis of human neurodevelopmental
and neurodegenerative diseases. Finally, we provide an
overview of the main challenges to overcome in order to
generate more physiologically relevant human SpC models and
propose some exciting new perspectives.},
subtyp = {Review Article},
keywords = {development (Other) / in vitro disease modeling (Other) /
induced pluripotent stem cells (iPSCs) (Other) / motor
neuron (MN) (Other) / motor neuron diseases (MNDs) (Other) /
organoids (Other) / spinal cord (SpC) (Other) / spinal cord
organoids (SCOs) (Other)},
cin = {AG Rodriguez-Muela},
ddc = {570},
cid = {I:(DE-2719)1713001},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37374039},
pmc = {pmc:PMC10303776},
doi = {10.3390/life13061254},
url = {https://pub.dzne.de/record/258922},
}