Pérdida de células de Purkinje asociada con alteraciones ultraestructurales específicas en un modelo de ratón con expresión transgénica de ataxina-2 mutada

Autores/as

Palabras clave:

Ataxia, Ataxia Espinocerebelosa tipo-2, ataxina-2, ratones transgénicos, desórdenes poliglutamínicos, células de Purkinje

Resumen

Introducción: La expresión de la proteína ataxina-2 con un dominio poliglutamínico expandido en su extremo N-terminal es la causa de la Ataxia Espinocerebelosa tipo-2, afectando primariamente a las células de Purkinje cerebelosas. El estudio morfológico de esta población neuronal expresando la ataxina-2 mutada podría brindar información sobre los mecanismos patogénicos subyacentes al proceso de enfermedad y contribuir a la identificación de potenciales dianas terapéuticas.

Objetivo: Identificar cambios en la celularidad y ultraestructura de células de Purkinje asociados a la expresión de la ataxina-2 con expansiones poliglutamínicas.

Material y Métodos: Tres grupos de ratones transgénicos (F066) de dos meses, cinco meses y 12 meses de edad expresando ataxina-2 con expansiones poliglutamínicas y un grupo de ratones salvajes de 12 meses de edad, fueron estudiados. Láminas histológicas de la capa de células de Purkinje correspondientes a cada grupo de animales fueron analizadas por microscopía convencional y electrónica.

Resultados: Consistente con estudios previos, una significativa reducción (p<0,05) en la densidad de células de Purkinje fue observada en los ratones F066 a los cinco (14,82±2,61) y 12 (13,9±0,58) meses de edad comparado con el grupo control (23,77±0,46). De manera similar, alteraciones ultraestructurales consistentes en pérdida de crestas mitocondriales, dilatación del retículo endoplasmático y del complejo de Golgi, fueron observadas en los tres grupos de animales transgénicos.

Conclusiones: La expresión transgénica de la ataxina-2 con expansiones poliglutamínicas está asociada con degeneración progresiva y con daños ultraestructurales de las células de Purkinje en el modelo F066. En consecuencia, los resultados sugieren que la ataxina-2 con expansiones poliglutamínicas podría ejercer su efecto neurotóxico a través de alteraciones funcionales del retículo endoplasmático, complejo de Golgi y mitocondrias.

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Biografía del autor/a

Dany Amauris Cuello Almartales, Centro para la Investigación y Rehabilitación de Ataxias Hereditarias. Holguín, Cuba

The author declare no conflicts of interest.

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Publicado

2025-12-21

Cómo citar

1.
Cuello Almartales DA, Aguiar Santiago J, Fernández Masso JR, Falcón Cama V, Almaguer Mederos LE, Velázquez-Pérez LC. Pérdida de células de Purkinje asociada con alteraciones ultraestructurales específicas en un modelo de ratón con expresión transgénica de ataxina-2 mutada. Rev haban cienc méd [Internet]. 21 de diciembre de 2025 [citado 19 de enero de 2026];24:e5451. Disponible en: https://revhabanera.sld.cu/index.php/rhab/article/view/5451

Número

Volumen 24 (2025)

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Ciencias Básicas Biomédicas

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