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• W2560651567 endingPage "e1006450" @default.
• W2560651567 startingPage "e1006450" @default.
• W2560651567 abstract "Misfolded proteins in transgenic models of conformational diseases interfere with proteostasis machinery and compromise the function of many structurally and functionally unrelated metastable proteins. This collateral damage to cellular proteins has been termed 'bystander' mechanism. How a single misfolded protein overwhelms the proteostasis, and how broadly-expressed mutant proteins cause cell type-selective phenotypes in disease are open questions. We tested the gain-of-function mechanism of a R37C folding mutation in an endogenous IGF-like C.elegans protein DAF-28. DAF-28(R37C) is broadly expressed, but only causes dysfunction in one specific neuron, ASI, leading to a distinct developmental phenotype. We find that this phenotype is caused by selective disruption of normal biogenesis of an unrelated endogenous protein, DAF-7/TGF-β. The combined deficiency of DAF-28 and DAF-7 biogenesis, but not of DAF-28 alone, explains the gain-of-function phenotype-deficient pro-growth signaling by the ASI neuron. Using functional, fluorescently-tagged protein, we find that, in animals with mutant DAF-28/IGF, the wild-type DAF-7/TGF-β is mislocalized to and accumulates in the proximal axon of the ASI neuron. Activation of two different branches of the unfolded protein response can modulate both the developmental phenotype and DAF-7 mislocalization in DAF-28(R37C) animals, but appear to act through divergent mechanisms. Our finding that bystander targeting of TGF-β explains the phenotype caused by a folding mutation in an IGF-like protein suggests that, in conformational diseases, bystander misfolding may specify the distinct phenotypes caused by different folding mutations." @default.
• W2560651567 created "2016-12-16" @default.
• W2560651567 creator A5008320600 @default.
• W2560651567 creator A5016205407 @default.
• W2560651567 creator A5067520165 @default.
• W2560651567 creator A5078640309 @default.
• W2560651567 date "2016-12-07" @default.
• W2560651567 modified "2023-10-13" @default.
• W2560651567 title "A Bystander Mechanism Explains the Specific Phenotype of a Broadly Expressed Misfolded Protein" @default.
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• W2560651567 doi "https://doi.org/10.1371/journal.pgen.1006450" @default.
• W2560651567 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5142776" @default.
• W2560651567 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/27926939" @default.
• W2560651567 hasPublicationYear "2016" @default.

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