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- W4386003773 abstract "Type 1 Charcot-Marie-Tooth disease (CMT1) is the most common demyelinating peripheral neuropathy. Patients suffer from progressive muscle weakness and sensory problems. The underlying disease mechanisms of CMT1 are still unclear and no therapy is currently available, hence patients completely rely on supportive care. Balancing protein levels is a complex multistep process fundamental to maintain cells in their healthy state and a disrupted proteostasis is a hallmark of several neurodegenerative diseases. When protein misfolding occurs, protein quality control systems are activated such as chaperones, the lysosomal-autophagy system and proteasomal degradation to ensure proper degradation. However, in pathological circumstances, these mechanisms are overloaded and thereby become inefficient to clear the load of misfolded proteins. Recent evidence strongly indicates that a disbalance in proteostasis plays an important role in several forms of CMT1. In this review, we present an overview of the protein quality control systems, their role in CMT1, and potential treatment strategies to restore proteostasis." @default.
- W4386003773 created "2023-08-20" @default.
- W4386003773 creator A5024275136 @default.
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- W4386003773 creator A5034195360 @default.
- W4386003773 creator A5069828577 @default.
- W4386003773 creator A5092665765 @default.
- W4386003773 date "2023-10-01" @default.
- W4386003773 modified "2023-10-12" @default.
- W4386003773 title "Proteostasis plays an important role in demyelinating Charcot Marie Tooth disease" @default.
- W4386003773 cites W1480359191 @default.
- W4386003773 cites W1498310106 @default.
- W4386003773 cites W1503394109 @default.
- W4386003773 cites W1557488126 @default.
- W4386003773 cites W1575978940 @default.
- W4386003773 cites W1578056497 @default.
- W4386003773 cites W1603142270 @default.
- W4386003773 cites W1620862744 @default.
- W4386003773 cites W1655540037 @default.
- W4386003773 cites W1878909517 @default.
- W4386003773 cites W1962850894 @default.
- W4386003773 cites W1966526764 @default.
- W4386003773 cites W1967308780 @default.
- W4386003773 cites W1968242810 @default.
- W4386003773 cites W1968902065 @default.
- W4386003773 cites W1970272191 @default.
- W4386003773 cites W1970467755 @default.
- W4386003773 cites W1970522739 @default.
- W4386003773 cites W1971830677 @default.
- W4386003773 cites W1972761807 @default.
- W4386003773 cites W1972799710 @default.
- W4386003773 cites W1973457937 @default.
- W4386003773 cites W1973854653 @default.
- W4386003773 cites W1973897761 @default.
- W4386003773 cites W1974723031 @default.
- W4386003773 cites W1975014950 @default.
- W4386003773 cites W1977603207 @default.
- W4386003773 cites W1981480597 @default.
- W4386003773 cites W1982659220 @default.
- W4386003773 cites W1986737010 @default.
- W4386003773 cites W1987357236 @default.
- W4386003773 cites W1989289268 @default.
- W4386003773 cites W1989441840 @default.
- W4386003773 cites W1989533700 @default.
- W4386003773 cites W1990255744 @default.
- W4386003773 cites W1991048512 @default.
- W4386003773 cites W1991402624 @default.
- W4386003773 cites W1991973434 @default.
- W4386003773 cites W1992900472 @default.
- W4386003773 cites W1993737194 @default.
- W4386003773 cites W1998255355 @default.
- W4386003773 cites W1998425738 @default.
- W4386003773 cites W1999704300 @default.
- W4386003773 cites W2000053674 @default.
- W4386003773 cites W2000678720 @default.
- W4386003773 cites W2003181300 @default.
- W4386003773 cites W2006690805 @default.
- W4386003773 cites W2007890784 @default.
- W4386003773 cites W2017396706 @default.
- W4386003773 cites W2017408392 @default.
- W4386003773 cites W2022116468 @default.
- W4386003773 cites W2022645386 @default.
- W4386003773 cites W2022964430 @default.
- W4386003773 cites W2025933441 @default.
- W4386003773 cites W2027485328 @default.
- W4386003773 cites W2028954557 @default.
- W4386003773 cites W2031565698 @default.
- W4386003773 cites W2032297609 @default.
- W4386003773 cites W2034994975 @default.
- W4386003773 cites W2035327540 @default.
- W4386003773 cites W2035863659 @default.
- W4386003773 cites W2036333455 @default.
- W4386003773 cites W2037089513 @default.
- W4386003773 cites W2038642910 @default.
- W4386003773 cites W2039949033 @default.
- W4386003773 cites W2041304851 @default.
- W4386003773 cites W2043823611 @default.
- W4386003773 cites W2044621729 @default.
- W4386003773 cites W2044826456 @default.
- W4386003773 cites W2047669413 @default.
- W4386003773 cites W2048384162 @default.
- W4386003773 cites W2049648491 @default.
- W4386003773 cites W2053048088 @default.
- W4386003773 cites W2053369913 @default.
- W4386003773 cites W2053885962 @default.
- W4386003773 cites W2055313891 @default.
- W4386003773 cites W2056433379 @default.
- W4386003773 cites W2058235401 @default.
- W4386003773 cites W2059524588 @default.
- W4386003773 cites W2061301855 @default.
- W4386003773 cites W2062473455 @default.
- W4386003773 cites W2065495697 @default.
- W4386003773 cites W2067061663 @default.
- W4386003773 cites W2067089499 @default.
- W4386003773 cites W2068275754 @default.
- W4386003773 cites W2072254697 @default.
- W4386003773 cites W2079224776 @default.
- W4386003773 cites W2082660003 @default.