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Deconstructing the Translational Control of Myelination by Specialized Ribosomes

Project description

Control of myelination and specialised ribosomes

Myelin is a lipid-based layer that surrounds nerve cell axons to insulate and increase the rate of electrical transduction along the axon. Myelin is formed by glial cells called oligodendrocytes in the central nervous system and Schwann cells in the peripheral nervous system. Myelination involves expansion of the glial cell membrane, which is the result of an increase in protein and lipid synthesis rates. There is emerging evidence that ribosomes are not just passive molecular machines and are heterogeneous in composition, and that particular ribosomal components might play a regulatory role of preferential translation of specific mRNAs. The EU-funded MyeRIBO project will employ advanced electron microscopy, quantitative proteomics, genome-wide ribosome profiling and genetic mouse models to investigate translation control by specialised ribosomes as a novel mechanism of regulation of myelination by the glial cells. MyeRIBO could have profound impact for understanding neural development and myelin disorders.

Objective

The myelin sheath is essential for neuronal function and health: myelinating glial cells speed up propagation of axonal potentials, fuel the energetic demands and regulate the ionic environment of neurons. Lesions to the myelin sheath thus result in devastating neurological disorders that include multiple sclerosis, diabetic neuropathy and Charcot-Marie-Tooth disease. Myelination involves a striking expansion of the glial cell membrane that relies on an exceptional increase in protein and lipid synthesis rates. Decades of dedicated research has uncovered a complex transcriptional program that drives this process, whereas translational control mechanisms, on the other hand, have received little attention. There is emerging evidence, enabled by modern techniques, that ribosomes, typically viewed as invariant, passive molecular machines, may instead be heterogeneous in composition, with particular ribosomal components having a specialized regulatory capacity for preferential translation of specific mRNAs. In MyeRIBO, I propose that translation control by specialized ribosomes is a novel layer of regulation that shapes the proteome of the myelinating glial cell. I will exploit advances in cryo-EM and quantitative proteomics analyses to discover the nature and diversity of ribosomes in myelinating cells, employ genome-wide ribosome profiling to obtain mechanistic insights into selective mRNA translation by heterogeneous ribosomes, and generate genetic mouse models to determine the functional consequences of this specialization for myelination in vivo. Notably, I will study the implication of this mechanism in pathogenesis of injury-induced demyelination and diabetic neuropathy, and evaluate the targeting of specialized ribosomal components as a preclinical strategy. MyeRIBO will push further the boundaries of our current understanding of the molecular control of myelination, which could have a profound impact for understanding neural development and myelin disorders.

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Keywords

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Programme(s)

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Topic(s)

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Funding Scheme

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ERC-COG - Consolidator Grant

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Call for proposal

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(opens in new window) ERC-2019-COG

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Host institution

UNIVERSIDAD DE SANTIAGO DE COMPOSTELA
Net EU contribution

Net EU financial contribution. The sum of money that the participant receives, deducted by the EU contribution to its linked third party. It considers the distribution of the EU financial contribution between direct beneficiaries of the project and other types of participants, like third-party participants.

€ 1 860 519,27
Address
COLEXIO DE SAN XEROME PRAZA DO OBRADOIRO S/N
15782 SANTIAGO DE COMPOSTELA
Spain

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Region
Noroeste Galicia A Coruña
Activity type
Higher or Secondary Education Establishments
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Total cost

The total costs incurred by this organisation to participate in the project, including direct and indirect costs. This amount is a subset of the overall project budget.

€ 1 860 519,27

Beneficiaries (2)

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