Inherited neurodegenerative diseases and transgenic models

Donald L. Price, Mark W. Becher, Philip C. Wong, David R. Borchelt, Michael K. Lee, Sangram S. Sisodia

Research output: Contribution to journalArticlepeer-review

6 Scopus citations


In recent years, the identification of mutations in specific genes in several inherited neurodegenerative disorders, combined with advances in the field of transgenic methods, has provided neuroscientists 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.

Original languageEnglish (US)
Pages (from-to)467-480
Number of pages14
JournalBrain Pathology
Issue number4
StatePublished - Oct 1996

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • General Neuroscience
  • Clinical Neurology


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