Proposed Pathway Underlying the Neurodegeneration Process in FTLF Spectrum Diseases (IMAGE)
Caption
Under normal physiological conditions, FUS and SFPQ interact in the nucleus of nerve cells and regulate the alternative splicing of MAPT by excising exon 10. When this functional machinery is impaired, the splicing ratio of MAPT exon 10+/exon 10- is increased, which in turn results in an increased 4R-Tau/3R-Tau ratio. The findings of Dr Ishigaki's team suggest the presence of a pathophysiological link between FUS/SFPQ interaction and the regulation of 4R-Tau/3R-Tau isoforms, which is involved in the pathogenesis of FTLD spectrum diseases.
Credit
Shinsuke Ishigaki
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