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Fibrillation and molecular characteristics are coherent with clinical and pathological features of 4-repeat tauopathy caused by MAPT variant G273R.

Abstract：

:Microtubule Associated Protein Tau (MAPT) forms proteopathic aggregates in several diseases. The G273R tau mutation, located in the first repeat region, was found by exome sequencing in a patient who presented with dementia and parkinsonism. We herein return to pathological examination which demonstrated tau immunoreactivity in neurons and glia consistent of mixed progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) features. To rationalize the pathological findings, we used molecular biophysics to characterize the mutation in more detail in vitro and in Drosophila. The G273R mutation increases the aggregation propensity of 4-repeat (4R) tau and alters the tau binding affinity towards microtubules (MTs) and F-actin. Tau aggregates in PSP and CBD are predominantly 4R tau. Our data suggest that the G273R mutation induces a shift in pool of 4R tau by lower F-actin affinity, alters the conformation of MT bound 4R tau, while increasing chaperoning of 3R tau by binding stronger to F-actin. The mutation augmented fibrillation of 4R tau initiation in vitro and in glial cells in Drosophila and showed preferential seeding of 4R tau in vitro suggestively causing a late onset 4R tauopathy reminiscent of PSP and CBD.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Sandberg A,Ling H,Gearing M,Dombroski B,Cantwell L,R'Bibo L,Levey A,Schellenberg GD,Hardy J,Wood N,Fernius J,Nyström S,Svensson S,Thor S,Hammarström P,Revesz T,Mok KY

doi

10.1016/j.nbd.2020.105079

subject

Has Abstract

pub_date

2020-12-01 00:00:00

pages

105079

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(20)30354-5

journal_volume

146

pub_type

杂志文章

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