DOI: 10.17151/biosa.2015.14.1.5
Cómo citar
Osorio, J. H. . (2015). Estudio In vitro de la deficiencia de Acil-CoA deshidrogenasa de cadena muy larga con afectación muscular. Biosalud, 14(1), 44–50. https://doi.org/10.17151/biosa.2015.14.1.5

Autores/as

José Henry Osorio
Universidad de Caldas
jose.osorio_o@ucaldas.edu.co

Resumen

Introducción: Los estudios in vitro, que buscan intermediarios de la degradación mitocondrial de ácidos grasos, facilitan el diagnóstico de alteraciones hereditarias o adquiridas en esa ruta metabólica. Objetivo: Analizar la producción de metabolitos en fibroblastos de pacientes con deficiencia de acil-CoA deshidrogenasa de cadena muy larga (VLCAD) con afectación muscular, incubados con ácido oleico deuterado. Materiales y Métodos: Se incubaron fibroblastos de tres pacientes con deficiencia de acil-CoA deshidrogenasa de cadena muy larga (VLCAD) con cardiomiopatía y de 10 controles en presencia de ácido oleico deuterado. Resultados: Se encontró un perfil característico luego de la incubación de fibroblastos con esta deficiencia. Conclusión: Este sustrato podría ser usado para realizar diagnóstico in vitro de la deficiencia de VLCAD con afectación muscular.

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