Composition of Rosenthal Fibers, the Protein Aggregate Hallmark of Alexander Disease.
作者： Michael R. Heaven , Daniel Flint , Shan M. Randall , Alexander A. Sosunov , Landon Wilson
DOI: 10.1021/ACS.JPROTEOME.6B00316
关键词:
摘要: Alexander disease (AxD) is a neurodegenerative disorder characterized by astrocytic protein aggregates called Rosenthal fibers (RFs). We used mouse models of AxD to determine the composition RFs obtain information about mechanisms including hypothesis that sequestration proteins in contributes disease. A method was developed for RF enrichment, and analysis resulting fraction using isobaric tags relative absolute quantitation mass spectrometry identified 77 not previously associated with RFs. Three five selected follow-up were confirmed enriched immunobloting both human patients: receptor activated C kinase 1 (RACK1), G1/S-specific cyclin D2, ATP-dependent RNA helicase DDX3X. Immunohistochemistry validated D2 as new component, but results RACK1 DDX3X equivocal. None these decreased non-RF fractions compared controls. simila...
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