. TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD. Nat Neurosci. 2018 Feb;21(2):228-239. Epub 2018 Jan 8 PubMed.


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  1. This paper from the Rossoll group is an important contribution to the emerging concept that disruption of nuclear architecture and nucleocytoplasmic transport are central mediators of neurodegeneration in multiple disorders. The nature of the nuclear envelope changes may differ somewhat among neurodegenerative disorders. We see that nuclear envelope aberrations in AD patient brain are quite clearly invaginations, whereas the nuclear envelope in ALS, FTD, and Huntington's disease patient material are typically described as "irregular," "ruffled," or "distorted." It is also important to determine if neurodegeneration causes general disruption of the nuclear envelope, versus active remodeling of the nuclear envelope in response to a stimulus. We've published evidence for the latter in the context of tauopathy.

    The overlap with work in the cancer field is interesting.

    View all comments by Bess Frost

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