Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome

Barth syndrome (BTHS) is a rare, X-linked cardiomyopathy that is characterized by abnormalities in glucose and lipid metabolism, with less known regarding amino acid metabolism. This pilot study characterized whole-body arginine kinetics and found lower arginine rate of appearance into plasma (0.69 ...

Descripción completa

Detalles Bibliográficos
Autores principales: Cade, W. Todd, Bohnert, Kathryn L., Bittel, Adam J., Chacko, Shaji J., Patterson, Bruce W., Pacak, Christina A., Byrne, Barry J., Vernon, Hilary J., Reeds, Dominic N.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Short Communication
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7649643/
https://www.ncbi.nlm.nih.gov/pubmed/33204638
http://dx.doi.org/10.1016/j.ymgmr.2020.100675
Descripción
Sumario:Barth syndrome (BTHS) is a rare, X-linked cardiomyopathy that is characterized by abnormalities in glucose and lipid metabolism, with less known regarding amino acid metabolism. This pilot study characterized whole-body arginine kinetics and found lower arginine rate of appearance into plasma (0.69 ± 0.09 vs. 0.88 ± 0.06 μmol/kgFFM/min, p < 0.01) and arginine non-oxidative disposal rate (0.64 ± 0.11 vs. 0.80 ± 0.03 μmol/kgFFM/min, p < 0.02) in adolescents and young adults with BTHS compared to Controls. This study provides a foundation for more in-depth studies on how arginine and potentially other amino acid abnormalities contribute to the pathology and clinical manifestations of BTHS.

Ejemplares similares