Wiskott-Aldrich syndrome protein (WASp) and relatives: A many-sided family
نویسندگان
چکیده
El síndrome de Wiskott-Aldrich (WAS) es una inmunodeficiencia primaria humana ligada al cromosoma X, caracterizada por inmunodeficiencia, microtrombocitopenia y eccema. El gen causante de WAS (WASP) codifica una proteína de 502 residuos llamada WASp. WASp da nombre a una familia de proteínas recientemente definida e involucrada en la transducción de señales desde la membrana celular al citoesqueleto de actina, así como en los procesos de desarrollo y activación celular. Además de su papel como organizador del citoesqueleto, WASp también es una proteína clave en la regulación de las señales procedentes de TCR/CD3 y que conducen a la activación transcripcional y proliferación de las células T. Además, WASp ha sido involucrada en la maduración de los timocitos. Así mismo, se ha mostrado que los miembros de la familia de WASp son importantes en el desarrollo de organismos multicelulares. Trabajos más recientes muestran que la familia WASp está involucrada también en diferentes enfermedades degenerativas. Esta revisión está centrada en la fisiología de la familia WASp, teniendo especial interés en la estructura de WASp, en la regulación intramolecular y en nuevos avances en terapia génica.
منابع مشابه
Protein-tyrosine kinase and GTPase signals cooperate to phosphorylate and activate Wiskott-Aldrich syndrome protein (WASP)/neuronal WASP.
Protein-tyrosine kinases and Rho GTPases regulate many cellular processes, including the reorganization and dynamics of the actin cytoskeleton. The Wiskott-Aldrich syndrome protein (WASP) and its homolog neuronal WASP (N-WASP) are effectors of the Rho GTPase Cdc42 and provide a direct link between activated membrane receptors and the actin cytoskeleton. WASP and N-WASP are also regulated by a l...
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