C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits
Abstract
A G4C2 repeat expansion in C9ORF72 is known to be the major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis (c9FTD/ALS). However, a lack of animal models recapitulating key disease features has hindered efforts to understand and prevent c9FTD/ALS-related neurodegeneration. Until now. Chew et al. describe a mouse model that mimics both neuropathological and clinical phenotypes of c9FTD/ALS.
Document Details
- Document Type
- Pub Defense Publication
- Publication Date
- Jun 05, 2015
- Source ID
- 10.1126/science.aaa9344
Entities
People
- Aishe Kurti
- Amelia Johnston
- Caroline Stetler
- Chris W. Lee
- Dennis W. Dickson
- Dieter Edbauer
- Emilie A. Perkerson
- Ena C. Whitelaw
- Hiroki Sasaguri
- Jeannette N. Stankowski
- Jeannie Chew
- John D. Fryer
- Karen Jansen-West
- Karen Overstreet
- Kevin B. Boylan
- Kevin F. Bieniek
- Leonard Petrucelli
- Lillian M. Daughrity
- Linda Rousseau
- Melissa E. Murray
- Mercedes Prudencio
- Monica Castanedes-Casey
- Pamela Desaro
- Peter O. Bauer
- Rosa Rademakers
- Tania F. Gendron
- Yong-Jie Zhang
Organizations
- ALS Association
- Alzheimer's Association
- German Center for Neurodegenerative Diseases
- Ludwig-Maximilians-Universität München
- Mayo Clinic
- Mayo Clinic Graduate School of Biomedical Sciences
- National Institute of Neurological Disorders and Stroke
- National Institute on Aging
- National Institutes of Health
- Seventh Framework Programme
- Target ALS
- United States Department of Defense