IndraLab

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USP19 increases the amount of PAH. 4 / 4
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"Overall, our results suggest that USP19 increases the cellular protein level of PAH variants by extending their half-lives, which results in increased Phe metabolism."
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"We found that ectopic expression of USP19 increased PAH protein level, whereas depletion of USP19 promoted PAH protein degradation."
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"Therefore, we initiated this study to investigate whether USP19, a previously identified DUB for PAHwt , can increase cellular levels of PAH variants through deubiquitinating activity.Notably, USP19 is an endoplasmic reticulum (ER)-specific DUB and participates in the export of misfolding-associated protein, which are associated with neurodegenerative diseases ."
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"Therefore, we investigated the functional consequence of USP19-mediated increase in the protein levels of the PAH variants on their metabolic function."
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