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La alfa B-cristalina se expresa en tejidos no lenticulares y se acumula en el cerebro de la enfermedad de Alexander

T Iwaki¹, A Kume-Iwaki, R K Liem

¹Department of Pathology, Columbia University College of Physicians and Surgeons, New York State Psychiatric Institute, New York 10032.

|April 7, 1989

Resumen

Las fibras de rosental en la enfermedad de Alejandro contienen alfa B-cristalina. Esta proteína, que normalmente se encuentra en la lente, también se acumula en los astrocitos durante las condiciones patológicas, lo que indica que no es específica de la lente.

Área de la Ciencia:

La neurociencia es la neurociencia.
Biología celular Biología celular.
Bioquímica de las proteínas Bioquímica de las proteínas

Sus antecedentes:

Las fibras de Rosenthal (RF) son inclusiones astrocíticas características en la enfermedad de Alexander.
Un componente proteico de 22 kDa de RFs fue previamente aislado del tejido cerebral de la enfermedad de Alexander.

Objetivo del estudio:

Para identificar el componente proteico de las fibras de Rosenthal.
Para investigar la distribución y la potencial especificidad no-lente de la alfa B-cristalina.

Principales métodos:

Western blotting para detectar equivalentes de proteínas en órganos de rata y una línea celular de astrocitoma humano.
Cribado de una biblioteca de ADNc con anticuerpos de proteínas anti-RF.
Pruebas inmunológicas para confirmar la reactividad de anticuerpos con alfa B-cristalina.

Principales resultados:

Se aisló un clon parcial de ADNc que codificaba la alfa B-cristalina de la línea celular del astrocitoma U-373MG.
Los anticuerpos de la proteína anti-RF reaccionaron con la lente alfa B-cristalina.
La alfa B-cristalina se detectó en varios órganos de rata, con los niveles más altos en el corazón y en la línea celular U-373MG.
La distribución del ARNm alfa B-cristalina en los órganos de las ratas reflejaba los hallazgos de las manchas occidentales.

Conclusiones:

La cristalina alfa B no es exclusivamente una proteína de lente.
La cristalina alfa B puede acumularse en los astrocitos bajo condiciones patológicas, formando fibras de Rosenthal.
Estos hallazgos contribuyen a la comprensión de la base molecular de la enfermedad de Alexander.

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