Impaired lipid metabolism in astrocytes underlies degeneration of cortical projection neurons in hereditary spastic paraplegia

Impaired lipid metabolism in astrocytes underlies degeneration of cortical projection neurons in hereditary spastic paraplegia

Abstract Hereditary spastic paraplegias (HSPs) are caused by a length-dependent axonopathy of long corticospinal neurons, but how axons of these cortical projection neurons (PNs) degenerate remains elusive. We generated isogenic human pluripotent stem cell (hPSC) lines for two ATL1 missense mutation...

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Bibliographic Details
Main Authors: Yongchao Mou, Yi Dong, Zhenyu Chen, Kyle R. Denton, Michael O. Duff, Craig Blackstone, Su-Chun Zhang, Xue-Jun Li
Format: Article
Language:English
Published: BMC 2020-12-01
Series:Acta Neuropathologica Communications
Subjects:
Hereditary spastic paraplegia
Human pluripotent stem cells
Cortical projection neurons
Cholesterol homeostasis
Axonal degeneration
Astrocytes
Online Access:https://doi.org/10.1186/s40478-020-01088-0

Internet

https://doi.org/10.1186/s40478-020-01088-0

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