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An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model
JoVE Journal
Genetica
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JoVE Journal Genetica
An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

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06:05 min

March 09, 2022

DOI:

06:05 min
March 09, 2022

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Summary

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Transmitochondrial cybrids are hybrid cells obtained by fusing mitochondrial DNA (mtDNA)-depleted cells (rho0 cells) with cytoplasts (enucleated cells) derived from patients affected by mitochondrial disorders. They allow the determination of the nuclear or mitochondrial origin of the disease, evaluation of biochemical activity, and confirmation of the pathogenetic role of mtDNA-related variants.

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