Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy.

By B. Ravikumar, R. Duden, D. Rubinsztein. Human Molecular Genetics, 2002

1,198 citations recorded by the source corpus.

Subfields: Cellular and Molecular Neuroscience, Epidemiology, Neurology

View Paper: https://www.semanticscholar.org/paper/b0fe2604a40569cae13a5af1456e1b7d00e970ab

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Citations in the corpus

Autophagy Inhibition Compromises Degradation of Ubiquitin-Proteasome Pathway Substrates (2009, neutral): “We also tested the effect of p62 overexpression on other polypeptides, such as mutant polyglutamine(polyQ)-expanded huntingtin fragment (EGFP-httQ74), mutanta-synuclein associated with familial Parkinson’s disease (EGFP-A53T), and EGFP alone, all of which are substrates for the UPS (Rubinsztein, 200”
Eaten alive: a history of macroautophagy (2010, background): “Early studies by Rubinsztein's laboratory demonstrated that autophagy affects the degradation of certain aggregate-prone proteins, such as those involved in Huntington's diseas”
Autophagy, amyloidogenesis and Alzheimer disease (2007, background): “presence of aggregates of mutant huntingtin, the pathogenic protein in Huntington disease (Ravikumar et al., 2002; Sarkar et al., 2005; Yamamoto et al., 2005; Shibata et al., 2006).”
CARGO RECOGNITION FAILURE IS RESPONSIBLE FOR INEFFICIENT AUTOPHAGY IN HUNTINGTON’S DISEASE (2010, background): “, contributes to the removal of both soluble and aggregate forms of mutant ht”
Rapamycin alleviates toxicity of different aggregate-prone proteins. (2006, background): “As we observed with mutant huntingtin constructs (14), rapamycin also decreased the levels of soluble ataxin1-Q92 relative to GFP (transfection control), whereas Baf A1 had the opposite effect (Fig.”
PI(5)P Regulates Autophagosome Biogenesis (2015, background): “We investigated the effects of PI5P4K2s knockdown and overexpression on the levels of diverse autophagic substrates, including the ubiquitin-binding adaptor protein p62 and a mutant form of huntingtin associated with Huntington’s disease (EGFP-httQ74) (Ravikumar et al., 2002).”
Autophagy gone awry in neurodegenerative diseases (2010, neutral): “20 ), including a growing number of neurological disorders such as Alzheimer's disease, Parkinson's disease, Huntington's disease and amyotrophic lateral sclerosis (ALS”
The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction. (2012, neutral): “In addition, a lack of autophagy completion in LSDs leads to the persistence of ubiquitinated and aggregate-prone polypeptides in the cytoplasm, including p62/SQSTM1, -synuclein, and Huntingtin protein (Ravikumar et al., 2002; Suzuki et al., 2007; Settembre et al., 2008; Tessitore et al., 2009).”