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1.
Mol Cell Biol ; 23(20): 7122-33, 2003 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-14517283

RESUMEN

The serine/threonine kinase PAK4 is a target for the Rho GTPase Cdc42 and has been shown to regulate cell morphology and cytoskeletal organization in mammalian cells. To examine the physiological and developmental functions of PAK4, we have disrupted the PAK4 gene in mice. The absence of PAK4 led to lethality by embryonic day 11.5, a result most likely due to a defect in the fetal heart. Striking abnormalities were also evident in the nervous systems of PAK4-deficient embryos. These embryos had dramatic defects in neuronal development and axonal outgrowth. In particular, spinal cord motor neurons and interneurons failed to differentiate and migrate to their proper positions. This is probably related to the role for PAK4 in the regulation of cytoskeletal organization and cell and/or extracellular matrix adhesion. PAK4-null embryos also had defects in proper folding of the caudal portion of the neural tube, suggesting an important role for PAK4 in neural tube development.


Asunto(s)
Neuronas/fisiología , Proteínas Serina-Treonina Quinasas/metabolismo , Proteínas Serina-Treonina Quinasas/fisiología , Proteína de Unión al GTP cdc42/metabolismo , Animales , Axones/metabolismo , Northern Blotting , Bromodesoxiuridina/farmacología , Adhesión Celular , Diferenciación Celular , Línea Celular , Movimiento Celular , Clonación Molecular , Citoesqueleto/metabolismo , ADN Complementario/metabolismo , Matriz Extracelular/metabolismo , Técnica del Anticuerpo Fluorescente Indirecta , Vectores Genéticos , Genotipo , Corazón/embriología , Humanos , Inmunohistoquímica , Etiquetado Corte-Fin in Situ , Ratones , Ratones Noqueados , Ratones Transgénicos , Modelos Genéticos , Cresta Neural/anomalías , Cresta Neural/citología , Cresta Neural/embriología , Neuronas/metabolismo , Neuronas/patología , Plásmidos/metabolismo , Reacción en Cadena de la Polimerasa , Médula Espinal/embriología , Factores de Tiempo , Distribución Tisular , Quinasas p21 Activadas
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