title: miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis.
abstract: Spinocerebellar ataxia type 1 is caused by expansion of a translated CAG repeat in ataxin1 (ATXN1). The level of the polyglutamine-expanded protein is one of the factors that contributes to disease severity. Here we found that miR-19, miR-101 and miR-130 co-regulate ataxin1 levels and that their inhibition enhanced the cytotoxicity of polyglutamine-expanded ATXN1 in human cells. We provide a new candidate mechanism for modulating the pathogenesis of neurodegenerative diseases sensitive to protein dosage.
authors.affiliation: Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA.
authors.forename: Yoontae
authors.initials: Y
authors.lastname: Lee
authors.forename: Rodney C
authors.initials: RC
authors.lastname: Samaco
authors.forename: Jennifer R
authors.initials: JR
authors.lastname: Gatchel
authors.forename: Christina
authors.initials: C
authors.lastname: Thaller
authors.forename: Harry T
authors.initials: HT
authors.lastname: Orr
authors.forename: Huda Y
authors.initials: HY
authors.lastname: Zoghbi