Matches in SemOpenAlex for { <https://semopenalex.org/work/W2017408392> ?p ?o ?g. }
- W2017408392 endingPage "164" @default.
- W2017408392 startingPage "153" @default.
- W2017408392 abstract "Charcot-Marie-Tooth disease type 1A (CMT1A) is commonly associated with duplication of the peripheral myelin protein 22 (PMP22) gene. Mice expressing seven copies of the human PMP22, termed C22, suffer from a demyelinating neuropathy and display phenotypic traits of CMT1A. In this article, we investigate whether protein aggregates play a role in the CMT1A-like pathology of C22 mice. Utilizing biochemical and immunochemical tools, we found slowed turnover rate of the newly-synthesized PMP22 and the presence of cytoplasmic protein aggregates in affected nerves. The formation of these aggregates correlates with reduced proteasome activity and the accumulation of detergent-insoluble ubiquitinated substrates. A fraction of the aggregates associates with autophagosomes and lysosomes. Together, these data indicate that as a result of missorting and inefficient proteasomal degradation, the aggregation of PMP22 and recruitment of autophagosomes and lysosomes are key factors in the subcellular pathogenesis of CMT1A neuropathies." @default.
- W2017408392 created "2016-06-24" @default.
- W2017408392 creator A5003020004 @default.
- W2017408392 creator A5037956505 @default.
- W2017408392 creator A5068252474 @default.
- W2017408392 creator A5069470638 @default.
- W2017408392 creator A5071211166 @default.
- W2017408392 creator A5079896790 @default.
- W2017408392 date "2006-04-01" @default.
- W2017408392 modified "2023-10-17" @default.
- W2017408392 title "Alterations in degradative pathways and protein aggregation in a neuropathy model based on PMP22 overexpression" @default.
- W2017408392 cites W1480359191 @default.
- W2017408392 cites W1530397717 @default.
- W2017408392 cites W1557488126 @default.
- W2017408392 cites W1570610869 @default.
- W2017408392 cites W1589854409 @default.
- W2017408392 cites W1620862744 @default.
- W2017408392 cites W1964982646 @default.
- W2017408392 cites W1968242810 @default.
- W2017408392 cites W1970522739 @default.
- W2017408392 cites W1972799710 @default.
- W2017408392 cites W1990255744 @default.
- W2017408392 cites W1990956809 @default.
- W2017408392 cites W1997369314 @default.
- W2017408392 cites W1998425738 @default.
- W2017408392 cites W2002559713 @default.
- W2017408392 cites W2008093868 @default.
- W2017408392 cites W2008141517 @default.
- W2017408392 cites W2011586442 @default.
- W2017408392 cites W2018098403 @default.
- W2017408392 cites W2021242983 @default.
- W2017408392 cites W2026613496 @default.
- W2017408392 cites W2028954557 @default.
- W2017408392 cites W2039949033 @default.
- W2017408392 cites W2039966629 @default.
- W2017408392 cites W2040276736 @default.
- W2017408392 cites W2046426342 @default.
- W2017408392 cites W2047304481 @default.
- W2017408392 cites W2055263556 @default.
- W2017408392 cites W2060215125 @default.
- W2017408392 cites W2082917147 @default.
- W2017408392 cites W2087059823 @default.
- W2017408392 cites W2089773021 @default.
- W2017408392 cites W2097293027 @default.
- W2017408392 cites W2103256993 @default.
- W2017408392 cites W2108086395 @default.
- W2017408392 cites W2111567059 @default.
- W2017408392 cites W2114959061 @default.
- W2017408392 cites W2128158982 @default.
- W2017408392 cites W2134309097 @default.
- W2017408392 cites W2136709809 @default.
- W2017408392 cites W2143866689 @default.
- W2017408392 cites W2146887271 @default.
- W2017408392 cites W2149761960 @default.
- W2017408392 cites W2151902088 @default.
- W2017408392 cites W2152708819 @default.
- W2017408392 cites W2156951081 @default.
- W2017408392 cites W2159228290 @default.
- W2017408392 cites W2170854435 @default.
- W2017408392 doi "https://doi.org/10.1016/j.nbd.2005.10.010" @default.
- W2017408392 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16326107" @default.
- W2017408392 hasPublicationYear "2006" @default.
- W2017408392 type Work @default.
- W2017408392 sameAs 2017408392 @default.
- W2017408392 citedByCount "112" @default.
- W2017408392 countsByYear W20174083922012 @default.
- W2017408392 countsByYear W20174083922013 @default.
- W2017408392 countsByYear W20174083922014 @default.
- W2017408392 countsByYear W20174083922015 @default.
- W2017408392 countsByYear W20174083922016 @default.
- W2017408392 countsByYear W20174083922017 @default.
- W2017408392 countsByYear W20174083922018 @default.
- W2017408392 countsByYear W20174083922019 @default.
- W2017408392 countsByYear W20174083922020 @default.
- W2017408392 countsByYear W20174083922021 @default.
- W2017408392 countsByYear W20174083922022 @default.
- W2017408392 countsByYear W20174083922023 @default.
- W2017408392 crossrefType "journal-article" @default.
- W2017408392 hasAuthorship W2017408392A5003020004 @default.
- W2017408392 hasAuthorship W2017408392A5037956505 @default.
- W2017408392 hasAuthorship W2017408392A5068252474 @default.
- W2017408392 hasAuthorship W2017408392A5069470638 @default.
- W2017408392 hasAuthorship W2017408392A5071211166 @default.
- W2017408392 hasAuthorship W2017408392A5079896790 @default.
- W2017408392 hasConcept C104317684 @default.
- W2017408392 hasConcept C127716648 @default.
- W2017408392 hasConcept C136238340 @default.
- W2017408392 hasConcept C169760540 @default.
- W2017408392 hasConcept C190062978 @default.
- W2017408392 hasConcept C203014093 @default.
- W2017408392 hasConcept C25602115 @default.
- W2017408392 hasConcept C27740335 @default.
- W2017408392 hasConcept C2778609137 @default.
- W2017408392 hasConcept C2780942790 @default.
- W2017408392 hasConcept C2781006897 @default.
- W2017408392 hasConcept C529278444 @default.
- W2017408392 hasConcept C55493867 @default.
- W2017408392 hasConcept C7602840 @default.