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Neuromuscular organoids model spinal neuromuscular pathologies in C9orf72 amyotrophic lateral sclerosis

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Title: Neuromuscular organoids model spinal neuromuscular pathologies in C9orf72 amyotrophic lateral sclerosis
Authors: Chong Gao, Qinghua Shi, Xue Pan, Jiajia Chen, Yuhong Zhang, Jiali Lang, Shan Wen, Xiaodong Liu, Tian-Lin Cheng, Kai Lei
Source: Cell Reports, Vol 43, Iss 3, Pp 113892- (2024)
Publisher Information: Elsevier, 2024.
Publication Year: 2024
Collection: LCC:Biology (General)
Subject Terms: CP: Neuroscience, Biology (General), QH301-705.5
More Details: Summary: Hexanucleotide repeat expansions in the C9orf72 gene are the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. Due to the lack of trunk neuromuscular organoids (NMOs) from ALS patients’ induced pluripotent stem cells (iPSCs), an organoid system was missing to model the trunk spinal neuromuscular neurodegeneration. With the C9orf72 ALS patient-derived iPSCs and isogenic controls, we used an NMO system containing trunk spinal cord neural and peripheral muscular tissues to show that the ALS NMOs could model peripheral defects in ALS, including contraction weakness, neural denervation, and loss of Schwann cells. The neurons and astrocytes in ALS NMOs manifested the RNA foci and dipeptide repeat proteins. Acute treatment with the unfolded protein response inhibitor GSK2606414 increased the glutamatergic muscular contraction 2-fold and reduced the dipeptide repeat protein aggregation and autophagy. This study provides an organoid system for spinal neuromuscular pathologies in ALS and its application for drug testing.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 2211-1247
Relation: http://www.sciencedirect.com/science/article/pii/S2211124724002201; https://doaj.org/toc/2211-1247
DOI: 10.1016/j.celrep.2024.113892
Access URL: https://doaj.org/article/d5afd73e42e94dc1903123b4edaf126b
Accession Number: edsdoj.5afd73e42e94dc1903123b4edaf126b
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  Data: Neuromuscular organoids model spinal neuromuscular pathologies in C9orf72 amyotrophic lateral sclerosis
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  Data: <searchLink fieldCode="AR" term="%22Chong+Gao%22">Chong Gao</searchLink><br /><searchLink fieldCode="AR" term="%22Qinghua+Shi%22">Qinghua Shi</searchLink><br /><searchLink fieldCode="AR" term="%22Xue+Pan%22">Xue Pan</searchLink><br /><searchLink fieldCode="AR" term="%22Jiajia+Chen%22">Jiajia Chen</searchLink><br /><searchLink fieldCode="AR" term="%22Yuhong+Zhang%22">Yuhong Zhang</searchLink><br /><searchLink fieldCode="AR" term="%22Jiali+Lang%22">Jiali Lang</searchLink><br /><searchLink fieldCode="AR" term="%22Shan+Wen%22">Shan Wen</searchLink><br /><searchLink fieldCode="AR" term="%22Xiaodong+Liu%22">Xiaodong Liu</searchLink><br /><searchLink fieldCode="AR" term="%22Tian-Lin+Cheng%22">Tian-Lin Cheng</searchLink><br /><searchLink fieldCode="AR" term="%22Kai+Lei%22">Kai Lei</searchLink>
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  Data: Cell Reports, Vol 43, Iss 3, Pp 113892- (2024)
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  Data: Elsevier, 2024.
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  Data: 2024
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  Data: Summary: Hexanucleotide repeat expansions in the C9orf72 gene are the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. Due to the lack of trunk neuromuscular organoids (NMOs) from ALS patients’ induced pluripotent stem cells (iPSCs), an organoid system was missing to model the trunk spinal neuromuscular neurodegeneration. With the C9orf72 ALS patient-derived iPSCs and isogenic controls, we used an NMO system containing trunk spinal cord neural and peripheral muscular tissues to show that the ALS NMOs could model peripheral defects in ALS, including contraction weakness, neural denervation, and loss of Schwann cells. The neurons and astrocytes in ALS NMOs manifested the RNA foci and dipeptide repeat proteins. Acute treatment with the unfolded protein response inhibitor GSK2606414 increased the glutamatergic muscular contraction 2-fold and reduced the dipeptide repeat protein aggregation and autophagy. This study provides an organoid system for spinal neuromuscular pathologies in ALS and its application for drug testing.
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  Data: http://www.sciencedirect.com/science/article/pii/S2211124724002201; https://doaj.org/toc/2211-1247
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