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Defectos de fosforilación oxidativa en el hígado de pacientes con enfermedad de Wilson
1University Department of Clinical Neurosciences, Royal Free and University College Medical School, and Institute of Neurology, London, UK.
Lancet (London, England)
|September 12, 2000
Resumen
La enfermedad de Wilson (WD) implica la deposición de cobre y causa una grave disfunción mitocondrial en el hígado. Este defecto del metabolismo energético sugiere que el daño oxidativo es clave para WD, apoyando la investigación de la terapia antioxidante.
Área de la Ciencia:
- La bioquímica es la bioquímica.
- Biología celular Biología celular.
- Genética La genética.
Sus antecedentes:
- La enfermedad de Wilson (WD) es el resultado de mutaciones en una ATPasa de tipo P, que conduce a la acumulación de cobre en el hígado y el cerebro.
- La proteína WD, una ATPasa transportadora de cobre, funciona en el Golgi pero su papel en las mitocondrias no está claro.
- La disfunción mitocondrial y el daño oxidativo están implicados en la patogénesis de WD.
Objetivo del estudio:
- Para investigar la función mitocondrial y la actividad de la aconitasa en el tejido hepático de la enfermedad de Wilson.
- Para comparar los parámetros mitocondriales en pacientes con WD con controles sanos y pacientes sin WD.
Principales métodos:
- Análisis de las actividades de las enzimas mitocondriales (complejo I, II + III, IV) y la aconitasa en muestras de hígado.
- Comparación del tejido hepático de WD con muestras de pacientes de control y no-WD.
Principales resultados:
- Se observaron reducciones significativas en las actividades de las enzimas mitocondriales en los hígados de WD: complejo I (62%), complejo II + III (52%), complejo IV (33%) y aconitasa (71%).
- Estos defectos fueron independientes del tratamiento con penicillamina, la colestasis o el deterioro de la función sintética hepática.
- Se encontraron pruebas de disfunción mitocondrial severa en el tejido hepático de WD.
Conclusiones:
- La enfermedad de Wilson se caracteriza por un defecto en el metabolismo energético hepático.
- La acumulación de cobre mitocondrial probablemente media la formación de radicales libres y el daño oxidativo, lo que contribuye a WD.
- Los hallazgos apoyan una mayor investigación sobre las terapias antioxidantes para la enfermedad de Wilson.
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