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Quantitative Microproteomics Based Characterization of the Central and Peripheral Nervous System of a Mouse Model of Krabbe Disease

ABSTRACT: In-depth characterization of the proteome of the Twitcher mouse, a mouse model for Krabbe disease, to investigate the proteome changes in the Central and Peripheral Nervous System.We applied a TMT-based workflow to compare the proteomes of the corpus callosum, motor cortex and sciatic nerves of littermate Twitcher and wild-type mice.

INSTRUMENT(S): Orbitrap Fusion ETD

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Brain, Sciatic Nerve

DISEASE(S): Krabbe Disease

SUBMITTER: Davide Pellegrini

LAB HEAD: Liam A. McDonnell

PROVIDER: PXD010594 | Pride | 2019-04-04

REPOSITORIES: Pride

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Quantitative Microproteomics Based Characterization of the Central and Peripheral Nervous System of a Mouse Model of Krabbe Disease.

Pellegrini Davide D Del Grosso Ambra A Angella Lucia L Giordano Nadia N Dilillo Marialaura M Tonazzini Ilaria I Caleo Matteo M Cecchini Marco M McDonnell Liam A LA

Molecular & cellular proteomics : MCP 20190329 6

Krabbe disease is a rare, childhood lysosomal storage disorder caused by a deficiency of galactosylceramide beta-galactosidase (GALC). The major effect of GALC deficiency is the accumulation of psychosine in the nervous system and widespread degeneration of oligodendrocytes and Schwann cells, causing rapid demyelination. The molecular mechanisms of Krabbe disease are not yet fully elucidated and a definite cure is still missing. Here we report the first in-depth characterization of the proteome ...[more]

PMID: 30926673

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