[HTML][HTML] Aberrant assembly of RNA recognition motif 1 links to pathogenic conversion of TAR DNA-binding protein of 43 kDa (TDP-43)
A Shodai, T Morimura, A Ido, T Uchida, T Ayaki… - Journal of Biological …, 2013 - ASBMB
Aggregation of TAR DNA-binding protein of 43 kDa (TDP-43) is a pathological signature of
amyotrophic lateral sclerosis (ALS). Although accumulating evidence suggests the
involvement of RNA recognition motifs (RRMs) in TDP-43 proteinopathy, it remains unclear
how native TDP-43 is converted to pathogenic forms. To elucidate the role of homeostasis of
RRM1 structure in ALS pathogenesis, conformations of RRM1 under high pressure were
monitored by NMR. We first found that RRM1 was prone to aggregation and had three …
amyotrophic lateral sclerosis (ALS). Although accumulating evidence suggests the
involvement of RNA recognition motifs (RRMs) in TDP-43 proteinopathy, it remains unclear
how native TDP-43 is converted to pathogenic forms. To elucidate the role of homeostasis of
RRM1 structure in ALS pathogenesis, conformations of RRM1 under high pressure were
monitored by NMR. We first found that RRM1 was prone to aggregation and had three …