Work

Intranuclear Neuronal Inclusions: A Common Pathogenic Mechanism for Glutamine-Repeat Neurodegenerative Diseases?

Year: 1997

Type: article

Abstract: Eight progressive neurodegenerative disorders, including Huntington's disease (HD) and several forms of spinocerebellar ataxia (SCA; see Table 1), are currently known to be caused by expanding CAG-triplet repeats coding for polyglutamine (Ross 1995; Andrew et al. 1997; Gusella et al. 1997). The genes causing these degenerative disorders show no homology to each other outside of the glutamine repeat, and most are genes of unknown function. In each of these diseases, an overlapping but distinct set of cortical and subcortical regions is affected. However, although the genes for the disorders are expressed widely throughout the brain, and also in peripheral tissues, only certain populations of neurons degenerate. The mechanism for this selective neuronal death has remained puzzling.

Source: Neuron

DOI: 10.1016/s0896-6273(00)80405-5

Author: Christopher A. Ross

Institutions: Johns Hopkins University, Johns Hopkins Medicine

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: hybrid

Estimated APC paid: $10,400