Evaluating the Bioenergetics Health Index Ratio in Leigh Syndrome Fibroblasts to Understand Disease Severity

Evaluating the Bioenergetics Health Index Ratio in Leigh Syndrome Fibroblasts to Understand Disease Severity

Several pediatric mitochondrial disorders, including Leigh syndrome (LS), impact mitochondrial (mt) genetics, development, and metabolism, leading to complex pathologies and energy failure. The extent to which pathogenic mtDNA variants regulate disease severity in LS is currently not well understood...

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Bibliographic Details
Main Authors: Ajibola B. Bakare, Joseph Dean, Qun Chen, Vedant Thorat, Yimin Huang, Thomas LaFramboise, Edward J. Lesnefsky, Shilpa Iyer
Format: Article
Language:English
Published: MDPI AG 2021-09-01
Series:International Journal of Molecular Sciences
Subjects:
mitochondrial disorders
leigh syndrome
glycolysis
mitochondrial respiration
bioenergetics health index
Online Access:https://www.mdpi.com/1422-0067/22/19/10344

Internet

https://www.mdpi.com/1422-0067/22/19/10344

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