What question did this study set out to answer?

The research investigates the relationship between KIAA1324 protein levels and TDP-43 function in ALS and FTD.

February 13, 2026Open Access

Lost in translation: absence of KIAA1324/ELAPOR1 protein in pathological TDP-43-affected neurons in ALS/FTD

Key Points

The research investigates the relationship between KIAA1324 protein levels and TDP-43 function in ALS and FTD.
Analyzed post-mortem brain tissue for KIAA1324 mRNA and protein levels
Investigated the relationship between KIAA1324 and TDP-43 in pathological conditions
Identified the absence of KIAA1324 in neurons affected by TDP-43
Found an inverse relationship between KIAA1324 mRNA levels and TDP-43 function
Reported near complete absence of KIAA1324 protein in TDP-43 affected neurons
Highlighted KIAA1324 as a potential therapeutic target downstream of TDP-43 in ALS/FTD

Abstract

The clear inverse relationship between KIAA1324 mRNA levels and TDP-43 function, and the near complete absence of KIAA1324 protein from neurons with pathological TDP-43 in post-mortem brain tissue, suggests KIAA3142 function is impaired in TDP-43 proteinopathies. Therefore, in addition to there being various disease mechanisms implicated in ALS, and TDP-43 being a challenging disease target to restore, KIAA1324 emerges as another of the many targets downstream of TDP-43 that may need to be addressed to demonstrate a therapeutic effect in ALS/FTD.

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Cite This Study

Cao et al. (Tue,) studied this question.

synapsesocial.com/papers/698ebedd85a1ff6a93016368 https://doi.org/https://doi.org/10.1186/s40478-026-02237-7

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