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LMBR1L regula la linfopoyesis a través de la señalización de Wnt/β-catenina
Jin Huk Choi1, Xue Zhong1, William McAlpine1
1Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
Science (New York, N.Y.)
|May 11, 2019
Resumen
Una mutación en la región del gen tipo 1 de las extremidades (Lmbr1l) afecta el desarrollo de las células inmunes al interrumpir la regulación de la señalización de Wnt. La deficiencia de Lmbr1I causa defectos de las células T y apoptosis, destacando su papel crítico en la linfopoyesis.
Área de la Ciencia:
- Inmunología
- Biología molecular
- La genética
Sus antecedentes:
- La regulación precisa de la señalización Wnt es crucial para el desarrollo adecuado del sistema inmunológico.
- El gen similar a la región 1 de la extremidad (Lmbr1l) codifica una proteína de membrana con un papel desconocido en la inmunidad.
Objetivo del estudio:
- Investigar la función de Lmbr1l en el desarrollo del sistema inmunológico.
- Para aclarar el mecanismo por el cual Lmbr1l regula la señalización de Wnt en los linfocitos.
Principales métodos:
- Inducción de mutaciones en ratones utilizando N-etil-N-nitrosourea para identificar genes que afectan el desarrollo linfoide.
- Análisis del desarrollo del linaje linfoide en ratones Lmbr1l mutantes.
- Investigación de las interacciones proteicas (LMBR1L, GP78, UBAC2) y su efecto sobre los componentes de la vía Wnt (FZD6, LRP6).
- Evaluación de la apoptosis de las células T y la activación de la vía Wnt/β-catenina en las células con deficiencia de Lmbr1l.
Principales resultados:
- Una mutación en Lmbr1l condujo a un desarrollo severamente deteriorado de todos los linajes linfoides en ratones.
- LMBR1L interactúa con GP78 y UBAC2 para atenuar la señalización de Wnt mediante la prevención de la maduración de FZD6 y LRP6 a través de la ubiquitinación y la estabilización de las proteínas complejas de destrucción.
- Las células T con deficiencia de LMBR1L mostraron signos de activación de Wnt/β-catenina y sufrieron apoptosis al recibir estímulos proliferativos.
Conclusiones:
- LMBR1L es esencial para la linfopoyesis y la activación linfoidea.
- LMBR1L funciona como un regulador negativo de la vía Wnt/β-catenina en el sistema inmune.
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