Dataset Information

Composition of Rosenthal fibers in Alexander disease patients and mouse models

ABSTRACT: Alexander disease (AxD) is a fatal neurodegenerative disorder characterized by the presence in astrocytes of protein aggregates called Rosenthal fibers (RFs). In this work, we used a new biochemical fractionation method to enrich for RFs followed by analysis of this fraction using quantitative iTRAQ mass spectrometry. This work identified 77 proteins not previously associated with RFs. Multiple proteins selected for follow-up were confirmed by immunobloting to be enriched in the RF fraction of both the AxD mouse models and human AxD patients including receptor for activated protein C kinase 1 (RACK1), G1/S-specific cyclin D2, and ATP-dependent RNA helicase DDX3X.

INSTRUMENT(S): TripleTOF 5600, Q Exactive

ORGANISM(S): Homo Sapiens (human) Mus Musculus (mouse)

TISSUE(S): Brain

SUBMITTER: Michael Brenner

LAB HEAD: Michael Brenner

PROVIDER: PXD002448 | Pride | 2016-05-23

REPOSITORIES: Pride

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Dataset's files

Source:

			Action	DRS
	Gel-1human.zip	Other
	Gel-1humanpeptideandproteinidentifications.xlsx	Xlsx
	Gel-2human.zip	Other
	Gel-2humanpeptideandproteinidentifications.xlsx	Xlsx
	ITRAQ_01_rep1.raw	Raw

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Publications

Composition of Rosenthal Fibers, the Protein Aggregate Hallmark of Alexander Disease.

Heaven Michael R MR Flint Daniel D Randall Shan M SM Sosunov Alexander A AA Wilson Landon L Barnes Stephen S Goldman James E JE Muddiman David C DC Brenner Michael M

Journal of proteome research 20160602 7

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 protein composition of RFs to obtain information about disease mechanisms including the hypothesis that sequestration of proteins in RFs contributes to disease. A method was developed for RF enrichment, and analysis of the resulting fraction using isobaric tags for relative and absolute quantitation mass spectrometry id ...[more]

PMID: 27193225

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