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Article

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

International journal of molecular sciences - 26 Sept 2021

Bakare Ajibola B, Dean Joseph, Chen Qun, Thorat Vedant, Huang Yimin, LaFramboise Thomas, Lesnefsky Edward J, Iyer Shilpa

Abstract excerpt

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. To better understand this relationship, we computed a glycolytic bioenergetics health index (BHI) for measuring...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 34638685
DOI: 10.3390/ijms221910344
PMCID: PMC8508996

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