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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.