Spinal Cord Organoids to Study Motor Neuron Development and Disease

Author:

Buchner Felix1,Dokuzluoglu Zeynep1ORCID,Grass Tobias1,Rodriguez-Muela Natalia123ORCID

Affiliation:

1. German Center for Neurodegenerative Diseases, 01307 Dresden, Germany

2. Center for Regenerative Therapies Dresden, Technische Universität Dresden, 01307 Dresden, Germany

3. Max Planck Institute for Molecular Cell Biology and Genetics, 01307 Dresden, Germany

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.

Funder

European Research Council

German Society for Muscle Diseases

DZNE-Helmholtz

TU Dresden CRTD

MPI-CBG

Publisher

MDPI AG

Subject

Paleontology,Space and Planetary Science,General Biochemistry, Genetics and Molecular Biology,Ecology, Evolution, Behavior and Systematics

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