Loss of EAAT4 accounts for the initial hyper-excitability of Purkinje cells lacking b-III spectrin and that loss of GLAST appears to work synergistically to worsen motor deficits. When levels of both EAAT4 and GLAST are compromised in b-III(-/-) mice the proximal dendrites of Purkinje cells within the posterior cerebellum are the most vulnerable to degeneration.
Study shows a direct interaction between beta-III spectrin and erythroid ankyrin in the cerebellum and to demonstrate a critical role for beta-III spectrin in maintaining ankyrin R throughout the Purkinje cell dendritic tree.
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