Polyglutamine Expansion Alters the Dynamics and Molecular Architecture of Aggregates in Dentatorubropallidoluysian Atrophy

Preferential accumulation of mutant proteins in the nucleus has been suggested to be the molecular culprit that confers cellular toxicity in the neurodegenerative disorders caused by polyglutamine (polyQ) expansion. Here, we use dynamic imaging approaches, orthogonal cross-seeding, and composition a...

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Veröffentlicht in:	The Journal of biological chemistry 2012-01, Vol.287 (3), p.2068-2078
Hauptverfasser:	Hinz, Justyna, Lehnhardt, Lothar, Zakrzewski, Silke, Zhang, Gong, Ignatova, Zoya
Format:	Artikel
Sprache:	eng
Schlagworte:	Aggregation Amyloid Animals Atrophin-1 Atrophy - genetics Atrophy - metabolism Atrophy - pathology Biophysics Cell Line Cross-seeding Cytoplasm - genetics Cytoplasm - metabolism Cytoplasm - pathology DRPLA Heredodegenerative Disorders, Nervous System - genetics Heredodegenerative Disorders, Nervous System - metabolism Heredodegenerative Disorders, Nervous System - pathology Humans Mice Microscopy Molecular Biophysics Multiprotein Complexes - genetics Multiprotein Complexes - metabolism Nerve Tissue Proteins - genetics Nerve Tissue Proteins - metabolism Peptides - genetics Peptides - metabolism Polyglutamine Polyglutamine Disease
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