The proteostasis guardian HSF1 directs the transcription of its paralog and interactor HSF2 during proteasome dysfunction
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DOI: 10.1007/s00018-020-03568-x
Abstract: Protein homeostasis is essential for life in eukaryotes. Organisms respond to proteotoxic stress by activating heat shock transcription factors (HSFs), which play important roles in cytoprotection, longevity and development. Of six human HSFs, HSF1 acts… read more here.
Keywords: transcription; hsf1; proteasome dysfunction; expression ... See more keywords
Autophagy activation can partially rescue proteasome dysfunction‐mediated cardiac toxicity
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DOI: 10.1111/acel.13715
Abstract: The ubiquitin–proteasome pathway and its functional interplay with other proteostatic and/or mitostatic modules are crucial for cell viability, especially in post‐mitotic cells like cardiomyocytes, which are constantly exposed to proteotoxic, metabolic, and mechanical stress. Consistently,… read more here.
Keywords: toxicity; dysfunction; heart targeted; proteasome dysfunction ... See more keywords
Report of the first patient with a homozygous OTUD7A variant responsible for epileptic encephalopathy and related proteasome dysfunction
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DOI: 10.1111/cge.13709
Abstract: Heterozygous microdeletions of chromosome 15q13.3 (MIM: 612001) show incomplete penetrance and are associated with a highly variable phenotype that may include intellectual disability, epilepsy, facial dysmorphism and digit anomalies. Rare patients carrying homozygous deletions show… read more here.
Keywords: epileptic encephalopathy; homozygous otud7a; dysfunction; proteasome dysfunction ... See more keywords
Collateral deletion of the mitochondrial AAA+ ATPase ATAD1 sensitizes cancer cells to proteasome dysfunction
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DOI: 10.7554/elife.82860
Abstract: The tumor suppressor gene PTEN is the second most commonly deleted gene in cancer. Such deletions often include portions of the chromosome 10q23 locus beyond the bounds of PTEN itself, which frequently disrupts adjacent genes.… read more here.
Keywords: cancer; deletion; atad1; proteasome dysfunction ... See more keywords