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Baylor College of Medicine

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Nat. Neurosci., Published online: 31 August 2008 | doi:10.1038/nn.2183 miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis

Abstract

Yoontae Lee^1,4, Rodney C Samaco¹, Jennifer R Gatchel², Christina Thaller³, Harry T Orr⁵ & Huda Y Zoghbi^1,2,3,4

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.

1. Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA.
2. Department of Neuroscience, Baylor College of Medicine, Houston, Texas 77030, USA.
3. Departments of Biochemistry and Molecular Biology, Baylor College of Medicine, Houston, Texas 77030, USA.
4. Howard Hughes Medical Institute, Baylor College of Medicine, Houston, Texas 77030, USA.
5. Institute of Human Genetics, Department of Biochemistry, Biophysics and Molecular Biology, Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota 55455, USA.

Correspondence to: Huda Y Zoghbi^1,2,3,4

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