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Article

Posterior cerebellar Purkinje cells in an SCA5/SPARCA1 mouse model are especially vulnerable to the synergistic effect of loss of β-III spectrin and GLAST.

Human molecular genetics - 15 Oct 2016

Perkins Emma M, Suminaite Daumante, Clarkson Yvonne L, Lee Sin Kwan, Lyndon Alastair R, Rothstein Jeffrey D, Wyllie David J A, Tanaka Kohichi, Jackson Mandy

Abstract excerpt

Clinical phenotypes of spinocerebellar ataxia type-5 (SCA5) and spectrin-associated autosomal recessive cerebellar ataxia type-1 (SPARCA1) are mirrored in mice lacking β-III spectrin (β-III-/-). One function of β-III spectrin is the stabilization of the Purkinje cell-specific glutamate transporter EAAT4 at the plasma membrane. In β-III-/- mice EAAT4 levels are reduced from an early age. In contrast levels of the...

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Identifiers

PMID: 28173092
DOI: 10.1093/hmg/ddw274
PMCID: PMC5409221

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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