Related Experiment Video
Updated: May 13, 2026

Symmetric Bihemispheric Postmortem Brain Cutting to Study Healthy and Pathological Brain Conditions in Humans
Published on: December 18, 2016
The unstable repeats--three evolving faces of neurological disease
David L Nelson1, Harry T Orr, Stephen T Warren
1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA. nelson@bcm.edu
Abstract:
Disorders characterized by expansion of an unstable nucleotide repeat account for a number of inherited neurological diseases. Here, we review examples of unstable repeat disorders that nicely illustrate three of the major pathogenic mechanisms associated with these diseases: loss of function typically by disrupting transcription of the mutated gene, RNA toxic gain of function, and protein toxic gain of function. In addition to providing insight into the mechanisms underlying these devastating neurological disorders, the study of these unstable microsatellite repeat disorders has provided insight into very basic aspects of neuroscience.
Related Concept Videos
Disorders of the Nervous Tissue
Homeostatic Imbalances:
Alzheimer's disease manifests as a gradual decline in memory and cognitive abilities, attributed to the buildup of amyloid plaques and neurofibrillary tangles in the brain.
Parkinson's disease arises from the...
Alzheimer Disease ll: Pathophysiology
Huntington Disease l: Introduction
Alzheimer Disease l: Introduction
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...
Traumatic Brain Injury l: Introduction

