Overview

Alexander disease


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Rare and usually fatal neurodegenerative disorder characterized by the development of megalencephaly in infancy accompanied by progressive spasticity and dementia. The features are similar to those of Canavan's disease. It is caused by a mutation in the glial fibrillary acidic protein (GFAP) gene. Astrocytes in Alexander's disease have protein aggregates (Rosenthal fibres) containing (alpha B-crystallin, heat shock protein 27, and GFAP.

Subjects: Chemistry — Medicine and Health.


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