Article
A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes.
Molecular neurobiology - 1 Jan 2022
Haas Eva, Incebacak Rana D, Hentrich Thomas, Huridou Chrisovalantou, Schmidt Thorsten, Casadei Nicolas, Maringer Yacine, Bahl Carola, Zimmermann Frank, Mills James D, Aronica Eleonora, Riess Olaf, Schulze-Hentrich Julia M, Hübener-Schmid Jeannette
Abstract excerpt
Spinocerebellar ataxia type 3 is the most common autosomal dominant inherited ataxia worldwide, caused by a CAG repeat expansion in the Ataxin-3 gene resulting in a polyglutamine (polyQ)-expansion in the corresponding protein. The disease is characterized by neuropathological, phenotypical, and specific transcriptional changes in affected brain regions. So far, there is no mouse model available representing all...
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