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Quantitative Magnetic Resonance Imaging of Skeletal Muscle Disease

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Published on: December 18, 2016

Una tormenta de SNO en el músculo esquelético

Jonathan S Stamler¹, Qi-An Sun, Douglas T Hess

¹Department of Medicine, Duke University Medical Center, Durham, NC 27710, USA. staml001@mc.duke.edu

|April 9, 2008

Resumen

La hiper-S-nitrosilación del canal de liberación de calcio del receptor de ryanodina (RyR1) en el músculo esquelético interrumpe el flujo de calcio. Este hallazgo explica la disfunción muscular en el ejercicio extenuante y trastornos congénitos como la hipertermia maligna.

Área de la Ciencia:

La bioquímica es la bioquímica.
Biología Molecular Biología Molecular
Fisiología Fisiología Fisiología.

Sus antecedentes:

La desregulación de la S-nitrosilación está implicada en varias enfermedades humanas, pero su papel específico sigue sin estar claro.
La S-nitrosilación es una modificación post-traduccional que implica la adición de un grupo de óxido nítrico a los residuos de cisteína.

Objetivo del estudio:

Investigar el papel de la S-nitrosilación en la función y enfermedad del músculo esquelético.
Examinar el impacto de la S-nitrosilación en el canal de liberación de calcio del receptor de ryanodina (RyR1).

Principales métodos:

Análisis de los niveles de proteína S-nitrosilación en el músculo esquelético.
Investigación del flujo de iones de calcio a través del canal de liberación de calcio del receptor de ryanodina (RyR1).
Correlación de la S-nitrosilación con la contractilidad del músculo esquelético y el daño celular.

Principales resultados:

Se observó hiper-S-nitrosilación de RyR1 en el músculo esquelético.
Esta hiper-S-nitrosilación interrumpe el flujo normal de iones de calcio.
El deterioro de la contractilidad y el daño celular en el músculo esquelético están relacionados con esta interrupción.

Conclusiones:

La desregulación de la S-nitrosilación de RyR1 contribuye a la disfunción del músculo esquelético.
Este mecanismo es relevante para el ejercicio extenuante y los trastornos musculares congénitos como la hipertermia maligna.

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