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El receptor de autofagia del retículo endoplasmático TEX264 impulsa la diferenciación epidérmica y está desregulado

Christopher J Johnson, Afua Tiwaa, Arti Parihar

bioRxiv : the preprint server for biology

|September 4, 2025

Resumen

TEX264 impulsa la diferenciación epidérmica promoviendo la descomposición del retículo endoplasmático (RE) a través de la reticulofagia, un proceso de autofagia selectiva crucial para la formación de la barrera cutánea. Este hallazgo arroja luz sobre trastornos de la cornificación como la enfermedad de Darier.

Área de la Ciencia:

Biología celular
Dermatología
Investigación de la autofagia

Sus antecedentes:

La diferenciación de los queratinocitos forma la barrera epidérmica a través de un proceso catabólico poco conocido.
Los organelos y la descomposición nuclear son eventos clave en la cornificación de los queratinocitos.

Objetivo del estudio:

Elucidar los mecanismos moleculares de la degradación de los orgánulos durante la diferenciación de los queratinocitos.
Investigar el papel de TEX264 en el proceso de cornificación y la formación de la barrera epidérmica.

Principales métodos:

Imágenes confocales en vivo de la epidermis organotípica humana.
Análisis de la expresión de TEX264 bajo estrés ER y en queratinocitos diferenciados.
Estudios de nocaut genético y mutación funcional de TEX264.
Examen de las biopsias de los pacientes y modelos organotípicos de la enfermedad de Darier.

Principales resultados:

Durante la cornificación se observó la fragmentación del retículo endoplasmático (ER) y la envoltura lisosómica.
TEX264, un receptor de la reticulofagia, fue regulado al alza durante la cornificación y el estrés ER.
TEX264 promovió la fragmentación y eliminación de ER y el encogimiento nuclear en los queratinocitos.
La deficiencia de TEX264 interrumpió la maduración epidérmica y sus niveles aumentaron en la enfermedad de Darier.

Conclusiones:

TEX264 es un regulador clave de la diferenciación y la cornificación de los queratinocitos.
La reticulofagia, mediada por TEX264, es esencial para la eliminación ordenada de los orgánulos durante la formación de la barrera epidérmica.
La desregulación de TEX264 está implicada en trastornos genéticos de la cornificación relacionados con la disfunción ER.

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