Failure to degrade CAT-tailed proteins disrupts neuronal morphogenesis and cell survival

T Udagawa, M Seki, T Okuyama, S Adachi, T Natsume… - Cell Reports, 2021 - cell.com
T Udagawa, M Seki, T Okuyama, S Adachi, T Natsume, T Noguchi, A Matsuzawa, T Inada
Cell Reports, 2021cell.com
Ribosome-associated quality control (RQC) relieves stalled ribosomes and eliminates
potentially toxic nascent polypeptide chains (NCs) that can cause neurodegeneration.
During RQC, RQC2 modifies NCs with a C-terminal alanine and threonine (CAT) tail. CAT
tailing promotes ubiquitination of NCs for proteasomal degradation, while RQC failure in
budding yeast disrupts proteostasis via CAT-tailed NC aggregation. However, the CAT tail
and its cytotoxicity in mammals have remained largely uncharacterized. We demonstrate …
Summary
Ribosome-associated quality control (RQC) relieves stalled ribosomes and eliminates potentially toxic nascent polypeptide chains (NCs) that can cause neurodegeneration. During RQC, RQC2 modifies NCs with a C-terminal alanine and threonine (CAT) tail. CAT tailing promotes ubiquitination of NCs for proteasomal degradation, while RQC failure in budding yeast disrupts proteostasis via CAT-tailed NC aggregation. However, the CAT tail and its cytotoxicity in mammals have remained largely uncharacterized. We demonstrate that NEMF, a mammalian RQC2 homolog, modifies translation products of nonstop mRNAs, major erroneous mRNAs in mammals, with a C-terminal tail mainly composed of alanine with several other amino acids. Overproduction of nonstop mRNAs induces NC aggregation and caspase-3-dependent apoptosis and impairs neuronal morphogenesis, which are ameliorated by NEMF depletion. Moreover, we found that homopolymeric alanine tailing at least partially accounts for CAT-tail cytotoxicity. These findings explain the cytotoxicity of CAT-tailed NCs and demonstrate physiological significance of RQC on proper neuronal morphogenesis and cell survival.
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