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La ausencia del coactivador SRC-2 da como resultado una glucogenopatía que se asemeja a la enfermedad de Von Gierke
Atul R Chopra1, Jean-Francois Louet, Pradip Saha
1Department of Molecular and Cellular Biology, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030, USA.
Science (New York, N.Y.)
|November 29, 2008
Resumen
El coactivador de transcripción SRC-2 controla la producción de glucosa hepática mediante la regulación de la enzima G6Pasa. La deficiencia de SRC-2 en ratones imita a Von Gierke en su trabajo.
Área de la Ciencia:
- La bioquímica es la bioquímica.
- Biología Molecular Biología Molecular
- Genética La genética.
Sus antecedentes:
- La producción de glucosa hepática es vital para la función cerebral y la supervivencia.
- La glucosa-6-fosfatasa (G6Pase) es una enzima clave en la liberación de glucosa en el plasma.
- Las mutaciones en la G6Pasa causan la enfermedad de Von Gierke (enfermedad de almacenamiento de glucógeno-1a).
Objetivo del estudio:
- Para identificar los reguladores de la liberación de glucosa hepática en ayunas.
- Para investigar el papel del coactivador de transcripción SRC-2 en el metabolismo de la glucosa.
- Para aclarar el mecanismo por el cual SRC-2 controla la expresión de G6Pase.
Principales métodos:
- Investigó el papel de SRC-2 en la regulación de la liberación de glucosa hepática en ayunas.
- Se analizó la interacción directa de SRC-2 con el receptor nuclear huérfano RORalpha.
- Examinó los efectos de la ablación de SRC-2 (corpo entero y hepático específico) en modelos de ratón.
Principales resultados:
- SRC-2 fue identificado como un regulador de la liberación de glucosa hepática en ayunas.
- SRC-2 modula directamente la expresión de la G6Pasa como coactivador con RORalpha.
- La ablación de SRC-2 en ratones resultó en un fenotipo de la enfermedad de Von Gierke.
Conclusiones:
- SRC-2 es un regulador crítico de la expresión hepática de la G6Pasa.
- SRC-2 juega un papel crucial en el control de la producción de glucosa en mamíferos.
- SRC-2 es esencial para prevenir el fenotipo de la enfermedad de Von Gierke.
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