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• W2530112418 abstract "Chorea-acanthocytosis is one of the hereditary neurodegenerative disorders known as the neuroacanthocytoses. Chorea-acanthocytosis is characterized by circulating acanthocytes deficient in chorein, a protein of unknown function. We report here for the first time that chorea-acanthocytosis red cells are characterized by impaired autophagy, with cytoplasmic accumulation of active Lyn and of autophagy-related proteins Ulk1 and Atg7. In chorea-acanthocytosis erythrocytes, active Lyn is sequestered by HSP90-70 to form high-molecular-weight complexes that stabilize and protect Lyn from its proteasomal degradation, contributing to toxic Lyn accumulation. An interplay between accumulation of active Lyn and autophagy was found in chorea-acanthocytosis based on Lyn coimmunoprecipitation with Ulk1 and Atg7 and on the presence of Ulk1 in Lyn-containing high-molecular-weight complexes. In addition, chorein associated with Atg7 in healthy but not in chorea-acanthocytosis erythrocytes. Electron microscopy detected multivesicular bodies and membrane remnants only in circulating chorea-acanthocytosis red cells. In addition, reticulocyte-enriched chorea-acanthocytosis red cell fractions exhibited delayed clearance of mitochondria and lysosomes, further supporting the impairment of authophagic flux. Because autophagy is also important in erythropoiesis, we studied in vitro CD34+-derived erythroid precursors. In chorea-acanthocytosis, we found (1) dyserythropoiesis; (2) increased active Lyn; (3) accumulation of a marker of autophagic flux and autolysososme degradation; (4) accumlation of Lamp1, a lysosmal membrane protein, and LAMP1-positive aggregates; and (5) reduced clearance of lysosomes and mitochondria. Our results uncover in chorea-acanthocytosis erythroid cells an association between accumulation of active Lyn and impaired autophagy, suggesting a link between chorein and autophagic vesicle trafficking in erythroid maturation." @default.
• W2530112418 created "2016-10-21" @default.
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• W2530112418 date "2016-12-22" @default.
• W2530112418 modified "2023-10-18" @default.
• W2530112418 title "A new molecular link between defective autophagy and erythroid abnormalities in chorea-acanthocytosis" @default.
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• W2530112418 cites W1899503782 @default.
• W2530112418 cites W1928401453 @default.
• W2530112418 cites W1967046029 @default.
• W2530112418 cites W1968432606 @default.
• W2530112418 cites W1969125395 @default.
• W2530112418 cites W1972516624 @default.
• W2530112418 cites W1975119431 @default.
• W2530112418 cites W1978304263 @default.
• W2530112418 cites W1978413572 @default.
• W2530112418 cites W1981201486 @default.
• W2530112418 cites W1981263669 @default.
• W2530112418 cites W1993050837 @default.
• W2530112418 cites W1993950505 @default.
• W2530112418 cites W1994069035 @default.
• W2530112418 cites W1997309327 @default.
• W2530112418 cites W2000375722 @default.
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• W2530112418 cites W2001675597 @default.
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• W2530112418 cites W2036988959 @default.
• W2530112418 cites W2053557848 @default.
• W2530112418 cites W2056426987 @default.
• W2530112418 cites W2060800225 @default.
• W2530112418 cites W2061620817 @default.
• W2530112418 cites W2062916483 @default.
• W2530112418 cites W2066142272 @default.
• W2530112418 cites W2078184099 @default.
• W2530112418 cites W2080168607 @default.
• W2530112418 cites W2082390299 @default.
• W2530112418 cites W2083538044 @default.
• W2530112418 cites W2084988418 @default.
• W2530112418 cites W2094693414 @default.
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• W2530112418 cites W2112743424 @default.
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• W2530112418 cites W2114653343 @default.
• W2530112418 cites W2114883157 @default.
• W2530112418 cites W2123705280 @default.
• W2530112418 cites W2124077831 @default.
• W2530112418 cites W2126677834 @default.
• W2530112418 cites W2128162080 @default.
• W2530112418 cites W2128401926 @default.
• W2530112418 cites W2132629607 @default.
• W2530112418 cites W2134626310 @default.
• W2530112418 cites W2138417438 @default.
• W2530112418 cites W2148488190 @default.
• W2530112418 cites W2149270461 @default.
• W2530112418 cites W2152462290 @default.
• W2530112418 cites W2153255725 @default.
• W2530112418 cites W2154387556 @default.
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• W2530112418 cites W2163603192 @default.
• W2530112418 cites W2164945122 @default.
• W2530112418 cites W2165684037 @default.
• W2530112418 cites W2170421654 @default.
• W2530112418 cites W2170564629 @default.
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• W2530112418 doi "https://doi.org/10.1182/blood-2016-07-727321" @default.
• W2530112418 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5179337" @default.
• W2530112418 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/27742708" @default.
• W2530112418 hasPublicationYear "2016" @default.
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