Article
Liver-X-receptor agonists rescue axonal degeneration in SPG11-deficient neurons via regulating cholesterol trafficking.
Neurobiology of disease - 15 Oct 2023
Chai Eric, Chen Zhenyu, Mou Yongchao, Thakur Gitika, Zhan Weihai, Li Xue-Jun
Abstract excerpt
Spastic paraplegia type 11 (SPG11) is a common autosomal recessive form of hereditary spastic paraplegia (HSP) characterized by the degeneration of cortical motor neuron axons, leading to muscle spasticity and weakness. Impaired lipid trafficking is an emerging pathology in neurodegenerative diseases including SPG11, though its role in axonal degeneration of human SPG11 neurons remains unknown. Here, we...
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