Work

The Neuropathology of CAG Repeat Diseases: Review and Update of Genetic and Molecular Features

Year: 1997

Type: review

Abstract: Classification of inherited neurodegenerative diseases is increasingly based on their genetic features, which supplement, clarify, and sometimes replace the older clinical and pathologic schemata. This change has been particularly rapid and impressive for the CAG repeat disorders. In Huntington's disease, X-linked spinobulbar muscular atrophy, dentatorubropallidoluysian atrophy, and a series of autosomal dominant cerebellar atrophies, genetic advances have resolved many nosologic issues, and opened new avenues for exploration of pathogenesis. In this review, we summarize classic and current concepts in neuropathology of these CAG repeat diseases.

Source: Brain Pathology

DOI: 10.1111/j.1750-3639.1997.tb00893.x

Authors: Yves Robitaille, Iscia Lopes‐Cendes, Mark Becher, Guy Rouleau, Arthur W. Clark

Institutions: Université de Montréal, McGill University, Johns Hopkins University, Johns Hopkins Medicine, University of Calgary

Language: English

Topic: Genetic Neurodegenerative Diseases

Subfield: Cellular and Molecular Neuroscience

Field: Neuroscience

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: green

OpenAlex ID: W2092442034

FWCI: 1.258

Cites: 228

Cited by: 131

Related to: 10