C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits-Reference-Cited by-同舟云学术

C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits

Published:2015-06-05 Issue:6239 Volume:348 Page:1151-1154
ISSN:0036-8075
Container-title:Science
language:en
Short-container-title:Science

Author:

Chew Jeannie¹²,Gendron Tania F.¹,Prudencio Mercedes¹,Sasaguri Hiroki¹,Zhang Yong-Jie¹,Castanedes-Casey Monica¹,Lee Chris W.¹,Jansen-West Karen¹,Kurti Aishe¹,Murray Melissa E.¹,Bieniek Kevin F.¹²,Bauer Peter O.¹,Whitelaw Ena C.¹,Rousseau Linda¹,Stankowski Jeannette N.¹,Stetler Caroline¹,Daughrity Lillian M.¹,Perkerson Emilie A.¹,Desaro Pamela³,Johnston Amelia³,Overstreet Karen³,Edbauer Dieter⁴⁵⁶,Rademakers Rosa¹²,Boylan Kevin B.³,Dickson Dennis W.¹²,Fryer John D.¹²,Petrucelli Leonard¹²

Affiliation:

1. Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA.

2. Neurobiology of Disease Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.

3. Department of Neurology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA.

4. German Center for Neurodegenerative Diseases (DZNE) Munich, Feodor-Lynen-Strasse 17, 81337 Munich, Germany.

5. Institute for Metabolic Biochemistry, Ludwig-Maximilians University Munich, Feodor-Lynen-Strasse 17, 81337 Munich, Germany.

6. Munich Cluster of Systems Neurology (SyNergy), Munich, Germany.

Abstract

A mouse model for ALS 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. Science , this issue p. 1151

Funder

NIH

U.S. Department of Defense

National Institute on Aging

National Institute of Neurological Disorders and Stroke

Mayo Clinic Foundation

Mayo Clinic Center for Regenerative Medicine

Alzheimer's Association

ALS Association

European Union’s Seventh Framework Programme

National Institute of Environmental Health Services

Mayo Graduate School