Polyglutamine expansion neurodegenerative disease
Related Experiment Video
Updated: Sep 21, 2026

Growth Assays to Assess Polyglutamine Toxicity in Yeast
Published on: March 5, 2012
1National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892-1250, USA. kf@codon.nih.gov
Abstract:
Kennedy's disease was the first of eight neurodegenerative disorders found to be caused by expanded polyglutamine repeats. Each of these disorders is likely caused by a toxic gain of function in the disease gene product, often associated with inclusions of mutant protein in susceptible neurons. The mechanism of toxicity may involve sequestration and depletion of a polyglutamine-containing protein that is important to neuronal survival, such as CREB-binding protein. Recent insights into the biochemistry and cellular pathology of the polyglutamine expansion neurodegenerative diseases provide the opportunity for systematic drug screens and a rational approach to effective therapy.
Related Concept Videos
Amyloid Fibrils
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining, normally used to...
Translation
Alzheimer Disease ll: Pathophysiology
Parkinson Disease ll: Pathophysiology
Huntington Disease l: Introduction

