Open AccessInherited Neurodegenerative Diseases and Transgenic Models
Author(s) -
Price Donald L.,
Becher Mark W.,
Wong Philip C.,
Borchelt David R.,
Lee Michael K.,
Sisodia Sangram S.
Publication year - 1996
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/j.1750-3639.1996.tb00876.x
Subject(s) - transgene , genetically modified mouse , neuroscience , biology , genetics , cancer research , computational biology , medicine , gene
In recent years, the identification of mutations in specific genes in several inherited neurodegener‐ative disorders, combined with advances in the field of transgenic methods, has provided neurosci‐entists and neuropathologists with information and strategies to develop transgenic (Tg) models to study human diseases. These approaches have proved to be extraordinarily useful in modeling familial forms of amyotrophic lateral sclerosis (FALS) and Alzheimer's disease (FAD) and the spectrum of triplet‐repeat disorders. Investigations of these models have begun to provide new insights into the roles of disease‐specific mutant proteins and the pathogenic mechanisms of disease as well as opportunities to test therapeutic interventions.