Increased mtDNA Abundance and Improved Function in Human Barth Syndrome Patient Fibroblasts Following AAV-TAZ Gene Delivery

Barth syndrome (BTHS) is a rare, X-linked, mitochondrial disorder caused by mutations in the gene encoding tafazzin. BTHS results in cardiomyopathy, muscle fatigue, and neutropenia in patients. Tafazzin is responsible for remodeling cardiolipin, a key structural lipid of the inner mitochondrial memb...

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Detalles Bibliográficos
Autores principales: Suzuki-Hatano, Silveli, Sriramvenugopal, Mughil, Ramanathan, Manash, Soustek, Meghan, Byrne, Barry J., Cade, W. Todd, Kang, Peter B., Pacak, Christina A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6678701/
https://www.ncbi.nlm.nih.gov/pubmed/31336787
http://dx.doi.org/10.3390/ijms20143416

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