The proteostasis guardian HSF1 directs the transcription of its paralog and interactor HSF2 during proteasome dysfunction
Sign Up to like & getrecommendations! Published in 2020 at "Cellular and Molecular Life Sciences"
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
Sign Up to like & getrecommendations! Published in 2022 at "Aging Cell"
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
Sign Up to like & getrecommendations! Published in 2020 at "Clinical Genetics"
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
Mutations in nucleotide metabolism genes bypass proteasome defects in png-1/NGLY1-deficient Caenorhabditis elegans
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DOI: 10.1371/journal.pbio.3002720
Abstract: The conserved SKN-1A/Nrf1 transcription factor regulates the expression of proteasome subunit genes and is essential for maintenance of adequate proteasome function in animal development, aging, and stress responses. Unusual among transcription factors, SKN-1A/Nrf1 is a… read more here.
Keywords: proteasome dysfunction; skn nrf1; png ngly1; proteasome ... 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