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Lysosome proteomics in Niemann-Pick Type C disease model cells (CHOwt and NPC1-null cells)

ABSTRACT: Increasing evidence implicates lysosomal dysfunction in the pathogenesis of neurodegenerative diseases, including the rare inherited lysosomal storage disorders (LSDs) and the most common neurodegenerative diseases, such as Alzheimer’s and Parkinson’s disease (AD and PD). The goal of this work was to analyze whether there are changes in the lysosomal glycocalyx in a cellular model of a LSD Niemann-Pick type C disease (NPC). Using the ferrofluid nanoparticles we isolated lysosomal organelles from NPC1-null and CHOwt cells. Mass spectrometry identification of lysosomal proteins was performed in order to determine the enrichment efficiency for N-glycome profiling of the lysosomal glycocalyx in NPC disease cellular model.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Homo Sapiens (human)

DISEASE(S): Neurodegenerative Disease

SUBMITTER: Anita Horvatic

LAB HEAD: Silva Katusic Hecimovic

PROVIDER: PXD008438 | Pride | 2018-03-21

REPOSITORIES: Pride

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N-glycome of the Lysosomal Glycocalyx is Altered in Niemann-Pick Type C Disease (NPC) Model Cells.

Kosicek Marko M Gudelj Ivan I Horvatic Anita A Jovic Tanja T Vuckovic Frano F Lauc Gordan G Hecimovic Silva S

Molecular & cellular proteomics : MCP 20180124 4

Increasing evidence implicates lysosomal dysfunction in the pathogenesis of neurodegenerative diseases, including the rare inherited lysosomal storage disorders (LSDs) and the most common neurodegenerative diseases, such as Alzheimer's and Parkinson's disease (AD and PD). Although the triggers of the lysosomal impairment may involve the accumulated macromolecules or dysfunction of the lysosomal enzymes, the role of the lysosomal glycocalyx in the lysosomal (dys)function has not been studied. The ...[more]

PMID: 29367433

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