Dataset Information

Proteomic dissection of Vanishing white matter pathogenesis

ABSTRACT: We aimed at gaining more insight into the molecular basis of VWM pathogenesis. Therefore we investigated protein expression patterns in the 2b5ho mouse model using a data-independent mass spectrometry-based quantitative proteomic analysis. The proteome of 4 different brain regions was analyzed at different time points of disease progression. Brain regions were selected based on their regional vulnerability to VWM, and included the cerebellum, corpus callosum, cortex, and brainstem. Commonalities and differences in proteome changes between 2b5ho mouse and VWM patient brains were assessed to determine disease-relevant protein changes during disease development and progression.

INSTRUMENT(S): Orbitrap Exploris 480

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Cerebellum, Brain, Commissural Neuron, Brainstem, Cortex

DISEASE(S): Leukodystrophy

SUBMITTER: Peter Robert Mosen

LAB HEAD: Marianna Bugiani

PROVIDER: PXD043872 | Pride | 2024-06-16

REPOSITORIES: Pride

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			Action	DRS
	20220930_EX3_UM7_mosen002_SA_EXT02_SA_Cb-VVM-042-05.raw	Raw
	20220930_EX3_UM7_mosen002_SA_EXT02_SA_Cb-VVM-365-03-RPT.raw	Raw
	20220930_EX3_UM7_mosen002_SA_EXT02_SA_Cb-VVM-365-04.raw	Raw
	20220930_EX3_UM7_mosen002_SA_EXT02_SA_Cb-WT-120-01.raw	Raw
	20220930_EX3_UM7_mosen002_SA_EXT02_SA_Cb-WT-196-01.raw	Raw

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Proteomic dissection of vanishing white matter pathogenesis.

Man Jodie H K JHK Zarekiani Parand P Mosen Peter P de Kok Mike M Debets Donna O DO Breur Marjolein M Altelaar Maarten M van der Knaap Marjo S MS Bugiani Marianna M

Cellular and molecular life sciences : CMLS 20240524 1

Vanishing white matter (VWM) is a leukodystrophy caused by biallelic pathogenic variants in eukaryotic translation initiation factor 2B. To date, it remains unclear which factors contribute to VWM pathogenesis. Here, we investigated the basis of VWM pathogenesis using the 2b5ho mouse model. We first mapped the temporal proteome in the cerebellum, corpus callosum, cortex, and brainstem of 2b5ho and wild-type (WT) mice. Protein changes observed in 2b5ho mice were t ...[more]

PMID: 38789799

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