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Sox2 es necesario para el desarrollo de los órganos sensoriales en el oído interno de los mamíferos
Amy E Kiernan1, Anna L Pelling, Keith K H Leung
1MRC Institute of Hearing Research, University of Nottingham, Nottingham NG7 2RD, UK.
Nature
|April 23, 2005
Resumen
El factor de transcripción SOX2 es crucial para el desarrollo del oído interno, especificando el pelo sensorial y las células de soporte. Su ausencia o reducción en ratones mutantes conduce a graves déficits auditivos y de equilibrio.
Área de la Ciencia:
- Biología del desarrollo Biología del desarrollo.
- La neurociencia es la neurociencia.
- Genética La genética.
Sus antecedentes:
- Las células ciliadas sensoriales del oído interno y las células de apoyo son vitales para la audición y el equilibrio.
- Su especificación de linaje de progenitores comunes implica mecanismos moleculares poco conocidos.
Objetivo del estudio:
- Investigar el papel del factor de transcripción SOX2 en el desarrollo del oído interno y la diferenciación de las células sensoriales.
- Para dilucidar las bases moleculares de la audición y los trastornos del equilibrio en ratones mutantes específicos.
Principales métodos:
- Análisis de dos mutantes alelicos de ratón (Lcc y Ysb) con déficits distintos de audición y equilibrio.
- Examen de la morfología del oído interno y la diferenciación de las células sensoriales en ratones mutantes y de tipo salvaje.
- Evaluación de los patrones de expresión génica de SOX2 y Math1 (Atoh1) durante el desarrollo del oído interno.
Principales resultados:
- Los ratones Lcc/Lcc carecen de expresión de SOX2, lo que resulta en un establecimiento fallido del dominio prosensorial y una grave malformación del oído interno.
- Los ratones Ysb/Ysb exhiben una expresión reducida de SOX2, lo que lleva a un desarrollo anormal de células ciliadas.
- La expresión de Math1 está ausente en los mutantes Lcc, mientras que SOX2 está presente en los mutantes Math1, lo que indica que SOX2 actúa aguas arriba de Math1.
Conclusiones:
- SOX2 es esencial para el desarrollo del dominio prosensorial del oído interno y la diferenciación tanto de las células ciliadas como de las células de soporte.
- SOX2 funciona aguas arriba de Math1 en la jerarquía genética que controla el desarrollo de las células sensoriales del oído interno.
- La desregulación de SOX2 está directamente relacionada con trastornos congénitos de la audición y el equilibrio.
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