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Nuclear inclusions go missing from neurones in hereditary ataxias

Mystery surrounding the events leading to degeneration of neurones in hereditary ataxias has deepened, as an immunohistochemical study discloses complete absence of nuclear inclusions in Purkinje cells in human brain stems. At least two opposing explanations are possible.

Nuclear inclusions are of interest because they are usually common in Purkinje cells in the brain stem in 10 or so inherited neurodegenerative diseases and contain products of the CAG repeat sequences in the disease gene. The authors chose four diseases with different degrees of cerebellar degeneration and compared the extent of nuclear inclusions, for evidence of a common pathogenesis.

The results were perplexing. Nuclear inclusions positive for either of two gene products—ubiquitin or polyglutamine—were restricted to the dentate nucleus in three diseases—spinocerebellar ataxia (SCA) 1, SCA3, and dentatorubral pallidoluysian atrophy (DRPLA)—and Golgi cells in SCA1, SCA2, and DRPLA. None occurred in Purkinje cells from any source.

A link between nuclear inclusions . . . [Full text of this article]