Identification of novel polyglutamine-expanded aggregation species in spinal and bulbar muscular atrophy

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

Montserrat Arrasate, Siddhartha Mitra, Erik S. Schweitzer et al. · Nature · 2004 · 2K citations

Life Sciences, Neurobiology, Neuroscience +15

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

Joan S. Steffan, Aleksey Kazantsev, Olivera Spasić-Bošković et al. · Proceedings of the National Academy of Sciences · 2000 · 1.1K citations

Life Sciences, Genetics, Molecular Biology +17

Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

Heidi Olzscha, Sonya M. Schermann, Andreas C. Woerner et al. · Cell · 2011 · 708 citations · Full text

Life Sciences, Biochemistry, Cell Biology +14

Progressive Disruption of Cellular Protein Folding in Models of Polyglutamine Diseases

Tali Gidalevitz, Anat Ben‐Zvi, Kim H. Ho et al. · Science · 2006 · 651 citations

Life Sciences, Biochemistry, Molecular Biology +17

Testosterone Reduction Prevents Phenotypic Expression in a Transgenic Mouse Model of Spinal and Bulbar Muscular Atrophy

Masahisa Katsuno, Hiroaki Adachi, Akito Kume et al. · Neuron · 2002 · 480 citations · Full text

Medicine, Neurobiology, Neuroscience +16