Science (American Association for the Advancement of Science), 2006-10, Vol.314 (5796), p.130-133
- Neumann, Manuela
- Sampathu, Deepak M
- Kwong, Linda K
- Truax, Adam C
- Micsenyi, Matthew C
- Chou, Thomas T
- Bruce, Jennifer
- Schuck, Theresa
- Grossman, Murray
- Clark, Christopher M
- McCluskey, Leo F
- Miller, Bruce L
- Masliah, Eliezer
- Mackenzie, Ian R
- Feldman, Howard
- Feiden, Wolfgang
- Kretzschmar, Hans A
- Trojanowski, John Q
- Lee, Virginia M.-Y
2006
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- Autor(en) / Beteiligte
- Neumann, Manuela
- Sampathu, Deepak M
- Kwong, Linda K
- Truax, Adam C
- Micsenyi, Matthew C
- Chou, Thomas T
- Bruce, Jennifer
- Schuck, Theresa
- Grossman, Murray
- Clark, Christopher M
- McCluskey, Leo F
- Miller, Bruce L
- Masliah, Eliezer
- Mackenzie, Ian R
- Feldman, Howard
- Feiden, Wolfgang
- Kretzschmar, Hans A
- Trojanowski, John Q
- Lee, Virginia M.-Y
- Titel
- Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
- Ist Teil von
- Science (American Association for the Advancement of Science), 2006-10, Vol.314 (5796), p.130-133
- Ort / Verlag
- Washington, DC: American Association for the Advancement of Science
- Erscheinungsjahr
- 2006
- Quelle
- Science Online_科学在线
- Beschreibungen/Notizen
- 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.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 0036-8075eISSN: 1095-9203DOI: 10.1126/science.1134108Titel-ID: cdi_proquest_miscellaneous_68932272
- Format
- –
- Schlagworte
- Amino Acid Sequence, Amyotrophic lateral sclerosis, Amyotrophic Lateral Sclerosis - metabolism, Amyotrophic Lateral Sclerosis - pathology, Antibodies, Antibodies, Monoclonal, Biological and medical sciences, Brain Chemistry, Cerebral Cortex - chemistry, Cerebral Cortex - pathology, Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases, Dementia - genetics, Dementia - metabolism, Dementia - pathology, DNA-Binding Proteins - analysis, DNA-Binding Proteins - chemistry, DNA-Binding Proteins - genetics, DNA-Binding Proteins - immunology, Fluorescent Antibody Technique, Frontotemporal lobar degeneration, Hippocampus - chemistry, Hippocampus - pathology, Humans, Immunoblotting, Medical sciences, Molecular Sequence Data, Motor Neurons - chemistry, Motor Neurons - pathology, Nervous system diseases, Neurodegenerative diseases, Neurological disorders, Neurology, Neurons, Neurons - chemistry, Neurons - pathology, Pathology, Peptide Fragments - chemistry, Phosphorylation, Proteins, Spinal cord, Spinal Cord - chemistry, Spinal Cord - pathology, Ubiquitin - analysis, Ubiquitins