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- W1998809945 endingPage "595" @default.
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- W1998809945 abstract "Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare congenital leukodystrophy caused by mutations in the MLC1 gene that encodes a membrane protein of unknown function. In the brain MLC1 protein is mainly expressed in astrocyte end-feet, localizes in lipid rafts and associates with the dystrophin glycoprotein complex (DGC). Using pull-down and co-fractionation assays in cultured human and rat astrocytes, we show here that MLC1 intracellular domains pull-down the DGC proteins syntrophin, dystrobrevin, Kir4.1 and caveolin-1, the structural protein of caveolae, thereby supporting a role for DGC and caveolar structures in MLC1 function. By immunostaining and subcellular fractionation of cultured rat or human astrocytes treated with agents modulating caveolin-mediated trafficking, we demonstrate that MLC1 is also expressed in intracellular vesicles and endoplasmic reticulum and undergoes caveolae/raft-mediated endocytosis. Inhibition of endocytosis, cholesterol lowering and protein kinases A- and C-mediated MLC1 phosphorylation favour the expression of membrane-associated MLC1. Because pathological mutations prevent MLC1 membrane expression, the identification of substances regulating MLC1 intracellular trafficking is potentially relevant for the therapy of MLC." @default.
- W1998809945 created "2016-06-24" @default.
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- W1998809945 date "2010-03-01" @default.
- W1998809945 modified "2023-10-04" @default.
- W1998809945 title "MLC1 trafficking and membrane expression in astrocytes: Role of caveolin-1 and phosphorylation" @default.
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- W1998809945 cites W1969732948 @default.
- W1998809945 cites W1975162799 @default.
- W1998809945 cites W1977172179 @default.
- W1998809945 cites W1978241559 @default.
- W1998809945 cites W1982483260 @default.
- W1998809945 cites W1986767210 @default.
- W1998809945 cites W1988339417 @default.
- W1998809945 cites W1993233373 @default.
- W1998809945 cites W1995150314 @default.
- W1998809945 cites W1995608940 @default.
- W1998809945 cites W2000092485 @default.
- W1998809945 cites W2004630293 @default.
- W1998809945 cites W2007512974 @default.
- W1998809945 cites W2008660560 @default.
- W1998809945 cites W2011199674 @default.
- W1998809945 cites W2013788682 @default.
- W1998809945 cites W2015752928 @default.
- W1998809945 cites W2017394237 @default.
- W1998809945 cites W2023292378 @default.
- W1998809945 cites W2030727312 @default.
- W1998809945 cites W2033725243 @default.
- W1998809945 cites W2033794046 @default.
- W1998809945 cites W2034508297 @default.
- W1998809945 cites W2037065330 @default.
- W1998809945 cites W2037984086 @default.
- W1998809945 cites W2039290509 @default.
- W1998809945 cites W2040548499 @default.
- W1998809945 cites W2040651358 @default.
- W1998809945 cites W2044964737 @default.
- W1998809945 cites W2052488789 @default.
- W1998809945 cites W2053988697 @default.
- W1998809945 cites W2060669063 @default.
- W1998809945 cites W2061992397 @default.
- W1998809945 cites W2067368375 @default.
- W1998809945 cites W2068829489 @default.
- W1998809945 cites W2071368833 @default.
- W1998809945 cites W2072365896 @default.
- W1998809945 cites W2073296814 @default.
- W1998809945 cites W2076951578 @default.
- W1998809945 cites W2091707906 @default.
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- W1998809945 cites W2122403700 @default.
- W1998809945 cites W2123830444 @default.
- W1998809945 cites W2124635182 @default.
- W1998809945 cites W2126326200 @default.
- W1998809945 cites W2127036217 @default.
- W1998809945 cites W2133982632 @default.
- W1998809945 cites W2139319426 @default.
- W1998809945 cites W2141321445 @default.
- W1998809945 cites W2144760773 @default.
- W1998809945 cites W2146017809 @default.
- W1998809945 cites W2148599181 @default.
- W1998809945 cites W2153805966 @default.
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- W1998809945 doi "https://doi.org/10.1016/j.nbd.2009.11.008" @default.
- W1998809945 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/19931615" @default.
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