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Evaluation of cardiolipin nanodisks as lipid replacement therapy for Barth syndrome

Barth syndrome (BTHS) is a mitochondrial disorder characterized by cardiomyopathy and skeletal muscle weakness. Disease results from mutations in the tafazzin (TAZ) gene, encoding a phospholipid transacylase. Defective tafazzin activity results in an aberrant cardiolipin (CL) profile. The feasibilit...

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Detalles Bibliográficos
Autores principales: Ikon, Nikita, Hsu, Fong-Fu, Shearer, Jennifer, Forte, Trudy M., Ryan, Robert O.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Editorial Department of Journal of Biomedical Research 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5895564/
https://www.ncbi.nlm.nih.gov/pubmed/29336355
http://dx.doi.org/10.7555/JBR.32.20170094