首页|MSC-mediated mitochondrial transfer restores mitochondrial DNA and function in neural progenitor cells of Leber's hereditary optic neuropathy

MSC-mediated mitochondrial transfer restores mitochondrial DNA and function in neural progenitor cells of Leber's hereditary optic neuropathy

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Leber's hereditary optic neuropathy(LHON)is a debilitating mitochondrial disease associated with mutations in mitochondrial DNA(mtDNA).Unfortunately,the available treatment options for LHON patients are limited due to challenges in mitochondrial replacement.In our study,we reprogramming LHON urine cells into induced pluripotent stem cells(iPSCs)and differentiating them into neural progenitor cells(NPCs)and neurons for disease modeling.Our research revealed that LHON neurons exhibited significantly higher levels of mtDNA mutations and reduced mitochondrial function,confirming the disease phenotype.However,through co-culturing LHON iPSC-derived NPCs with mesenchymal stem cells(MSCs),we observed a remarkable rescue of mutant mtDNA and a significant improvement in mitochondrial metabolic function in LHON neurons.These findings suggest that co-culturing with MSCs can enhance mitochondrial function in LHON NPCs,even after their differentiation into neurons.This discovery holds promise as a potential therapeutic strategy for LHON patients.

mitochondriamitochondrial DNAmitochondrial diseasesinduced pluripotent stem cellsstem cellsmetabolismenergymesenchymal stem cells

Rui Wang、Feixiang Bao、Manjiao Lu、Xiaoyun Jia、Jiahui Xiao、Yi Wu、Qingjiong Zhang、Xingguo Liu

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Joint School of Life Sciences,Guangzhou Institutes of Biomedicine and Health,Chinese Academy of Sciences

Guangzhou Medical University,Guangzhou 510530,China

Centre for Regenerative Medicine and Health,Hong Kong Institute of Science & Innovation,Chinese Academy of Sciences,Hong Kong 99077,China

Guangdong Provincial Key Laboratory of Stem Cell and Regenerative Medicine,Guangdong-Hong Kong Joint Laboratory for Stem Cell and Regenerative Medicine,China-New Zealand Joint Laboratory on Biomedicine and Health,CUHK

Guangdong Provincial Key Laboratory of Stem Cell and Regenerative Medicine,Guangdong-Hong Kong Joint Laboratory for Stem Cell and Regenerative Medicine,China-New Zealand Joint Laboratory on Biomedicine and Health,CUHK-GIBH Joint Research Laboratory on Stem Cells and Regenerative Medicine,GIBH-HKU Guangdong-Hong Kong Stem Cell and Regenerative Medicine R

State Key Laboratory of Ophthalmology,Zhongshan Ophthalmic Center,Sun Yat-sen University,Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science,Guangzhou 510060,China

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2024

10.1007/s11427-024-2647-8

中国科学:生命科学(英文版)
中国科学院

中国科学:生命科学(英文版)

CSTPCD
影响因子：0.806
ISSN：1674-7305
年,卷(期)：2024.67(11)