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Spinocerebellar ataxia refers to a group of genetic, progressive neurodegenerative disorders of the spine and the cerebellum that can affect balance, coordination and speech.
Molecular changes in neurodegeneration occur much earlier than previously expected. In this study, dynamic molecular network analysis of iPSC differentiation uncovers a temporal pathway from histone to ISG15 with the earliest molecular changes of SCA1.
A recent study of clinical and genetic characteristics in patients with hereditary spastic paraplegia highlights the difficulties of making clinicogenetic correlations in a heterogeneous group of diseases. Genetic analysis beyond the causative variants, and independent of the core clinical symptoms (pyramidal signs versus ataxia), might offer a more pertinent way to approach phenotypic variability.
Neuronal atrophy early in a mouse model of spinal cerebellar ataxia 1 may represent an adaptive mechanism that restores the density of potassium channels in Purkinje neurons, thus normalizing membrane polarization and firing.
