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- W2989598512 endingPage "24650" @default.
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- W2989598512 abstract "Proteasome-mediated degradation of intracellular proteins is essential for cell function and survival. The proteasome-binding protein PI31 (Proteasomal Inhibitor of 31kD) promotes 26S assembly and functions as an adapter for proteasome transport in axons. As localized protein synthesis and degradation is especially critical in neurons, we generated a conditional loss of PI31 in spinal motor neurons (MNs) and cerebellar Purkinje cells (PCs). A cKO of PI31 in these neurons caused axon degeneration, neuronal loss, and progressive spinal and cerebellar neurological dysfunction. For both MNs and PCs, markers of proteotoxic stress preceded axonal degeneration and motor dysfunction, indicating a critical role for PI31 in neuronal homeostasis. The time course of the loss of MN and PC function in developing mouse central nervous system suggests a key role for PI31 in human neurodegenerative diseases." @default.
- W2989598512 created "2019-12-05" @default.
- W2989598512 creator A5019571251 @default.
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- W2989598512 creator A5064350961 @default.
- W2989598512 creator A5079762040 @default.
- W2989598512 creator A5083867613 @default.
- W2989598512 date "2019-11-21" @default.
- W2989598512 modified "2023-09-30" @default.
- W2989598512 title "The proteasome regulator PI31 is required for protein homeostasis, synapse maintenance, and neuronal survival in mice" @default.
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