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• Vol.52 No.11
• CLINICA NEUROL, 52: 1056−1058, 2012
• Vol.52 No.11 contents

Symposium 2

Intracellular seeded aggregation of TDP-43

Takashi Nonaka and Masato Hasegawa

Tokyo Metropolitan Institute of Medical Science

TAR-DNA binding protein of 43 kDa (TDP-43) is the component protein of inclusions in brains of patients with frontotemporal lobar degeneration (FTLD-TDP) and amyotrophic lateral sclerosis (ALS). Here we report a seed-dependent TDP-43 aggregation model using SH-SY5Y cells into which detergent-insoluble TDP-43 from diseased brains is introduced to provide seeds for aggregation. When these seeds were introduced into cells expressing HA-tagged TDP-43, round aggregates composed of phosphorylated and ubiquitinated HA-tagged TDP-43 were formed. Biochemical fractionation revealed the presence of Sarkosyl-insoluble phosphorylated full-length TDP-43 as well as its C-terminal fragments. Cells bearing TDP-43 inclusions exhibited increased levels of cell death and proteasome dysfunction. This seeding model reproduces characteristic features of affected neurons in brains with TDP-43 proteinopathy.
Full Text of this Article in Japanese PDF (216K)

(CLINICA NEUROL, 52: 1056−1058, 2012)
key words: frontotemporal lobar degeneration (FTLD-TDP) , amyotrophic lateral sclerosis (ALS), TAR-DNA binding protein of 43 kDa, propagation, seed-dependent protein aggregation

(Received: 24-May-12)