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El eje ERG-APLNR controla la proliferación endotelial de las venas pulmonares en la enfermedad pulmonar veno-oclusiva

Christopher Lathen¹, Yu Zhang¹, Jennifer Chow¹

¹From the Division of Cardiothoracic Surgery (C.L., Y.Z, J.C., M.S., Y.A.A., P.A.T), Department of Pathology (G.L.), Division of Cardiology (V.N), University of California, San Diego; Department of Medicine, University of Illinois, Chicago (J.X.Y.); and Division of Pulmonary Medicine, Vanderbilt University School of Medicine, Nashville, TN (I.M.R.).

|July 27, 2014

Resumen

La enfermedad pulmonar veno-oclusiva implica el daño de las venas pulmonares. Los investigadores encontraron que la vía Erg-Aplnr es crucial para la salud endotelial venosa y puede ser un objetivo terapéutico para esta condición incurable.

Área de la Ciencia:

Biología vascular Biología vascular
Genética molecular genética molecular.
Investigaciones cardiovasculares en la investigación cardiovascular.

Sus antecedentes:

La enfermedad pulmonar veno-oclusiva (PVOD) causa hipertensión pulmonar severa y insuficiencia cardíaca derecha debido a la obliteración de las venas pulmonares.
PVOD tiene un mal pronóstico con menos del 5% de supervivencia a 5 años y carece de tratamientos efectivos.
Comprender los mecanismos de las PVOD es fundamental para el desarrollo de nuevas terapias.

Objetivo del estudio:

Investigar el papel del factor de transcripción Ets Erg y sus objetivos aguas abajo en la patogénesis de la enfermedad pulmonar veno-oclusiva.
Identificar posibles dianas terapéuticas para el PVOD mediante el esclarecimiento de los mecanismos moleculares subyacentes.

Principales métodos:

Se utilizaron modelos de ratón con deleción homocigótica de Erg y Aplnr (receptor de la apelina).
Evaluar los fenotipos de la enfermedad pulmonar veno-oclusiva, incluida la mortalidad, la hemorragia y la proliferación endotelial.
Se analizaron los niveles de expresión de Erg y Aplnr en tejidos pulmonares de pacientes con PVOD.

Principales resultados:

La deficiencia de erg en ratones condujo a PVOD, hemorragia capilar y pancitopenia.
Erg activa directamente la expresión de Aplnr, que es específica para el endotelio venoso.
El nocaut de Erg o Aplnr indujo la proliferación endotelial de las venas pulmonares in vitro y el PVOD in vivo.
Se observaron niveles reducidos de ERG y APLNR en pacientes con PVOD.

Conclusiones:

ERG y APLNR son esenciales para mantener la homeostasis endotelial de las venas pulmonares.
La interrupción de la vía de señalización ERG-APLNR es un factor clave en el desarrollo de PVOD.
La vía ERG-APLNR representa un objetivo terapéutico prometedor para el tratamiento de la enfermedad pulmonar venooclusiva.

Palabras clave:

hipertensión arterial, hipertensión pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión arterial pulmonar, hipertensión pulmonar las venas pulmonares.enfermedad pulmonar veno-oclusiva pulmonar.

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