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Updated: Jun 16, 2025

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia
Published on: February 9, 2020
Generation of hereditary spastic paraplegia patient-derived induced pluripotent stem cell line UJSi003-A
Tingkai Sun1, Bonan Chen1, Dingqi Zhang1
1Neurobiology & Mitochondrial Key Laboratory, Effective & Toxicity Monitoring Innovative Practice Center for Food Pharmaceutical Specialty, School of Pharmacy, Jiangsu University, Zhenjiang 212013, China.
Abstract:
Hereditary spastic paraplegia (HSP) is a rare neurodegenerative disorder with the predominant clinical manifestation of spasticity in the lower extremities. Patients with HSP experience spastic paralysis in both lower limbs, leading to progressive walking difficulties, increased reflexes, spasms, and extensor plantar responses. We successfully generated induced pluripotent stem cells (iPSCs) from peripheral blood mononuclear cells (PBMCs) obtained from a patient diagnosed with HSP. The iPSCs exhibited a normal karyotype, expressed pluripotency markers, and differentiated into the three germ layers in vitro.
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