Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

Author:

Neumann Manuela12345,Sampathu Deepak M.12345,Kwong Linda K.12345,Truax Adam C.12345,Micsenyi Matthew C.12345,Chou Thomas T.12345,Bruce Jennifer12345,Schuck Theresa12345,Grossman Murray12345,Clark Christopher M.12345,McCluskey Leo F.12345,Miller Bruce L.12345,Masliah Eliezer12345,Mackenzie Ian R.12345,Feldman Howard12345,Feiden Wolfgang12345,Kretzschmar Hans A.12345,Trojanowski John Q.12345,Lee Virginia M.-Y.12345

Affiliation:

1. Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

2. Department of Pharmacology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

3. Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

4. Alzheimer's Disease Core Center, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

5. Institute on Aging, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

Abstract

Ubiquitin-positive, tau- and α-synuclein–negative inclusions are hallmarks of frontotemporal lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis. Although the identity of the ubiquitinated protein specific to either disorder was unknown, we showed that TDP-43 is the major disease protein in both disorders. Pathologic TDP-43 was hyper-phosphorylated, ubiquitinated, and cleaved to generate C-terminal fragments and was recovered only from affected central nervous system regions, including hippocampus, neocortex, and spinal cord. TDP-43 represents the common pathologic substrate linking these neurodegenerative disorders.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

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