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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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Detalles Bibliográficos
Autores principales: Bakare, Ajibola B., Dean, Joseph, Chen, Qun, Thorat, Vedant, Huang, Yimin, LaFramboise, Thomas, Lesnefsky, Edward J., Iyer, Shilpa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8508996/
https://www.ncbi.nlm.nih.gov/pubmed/34638685
http://dx.doi.org/10.3390/ijms221910344