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The Protein -glucosyltransferase Rumi Modifies Eyes Shut to Promote Rhabdomere Separation in


Glycosylation (addition of sugars to proteins and other organic molecules) is important for protein function and animal development. Each form of glycosylation is usually present on multiple proteins. Therefore, a major challenge in understanding the role of sugars in animal development is to identify which protein(s) modified by a specific sugar require the sugar modification for proper functionality. We have previously shown that an enzyme called Rumi adds glucose molecules to an important cell surface receptor called Notch, and that glucose plays a key role in the function of Notch both in fruit flies and in mammals. Using fruit flies, we have now identified a new Rumi target called “Eyes shut”, a secreted protein with a critical role in the optical isolation of neighboring photoreceptors in the fly eye. Our data suggest that glucose molecules on Eyes shut promote its folding and stability in a critical time window during eye development. Mutations in human Eyes shut result in a devastating form of retinal degeneration and loss of vision. Since human Eyes shut is also predicted to harbor glucose molecules, our work provides a framework to explore the role of sugar modifications in the biology of a human disease protein.


Vyšlo v časopise: The Protein -glucosyltransferase Rumi Modifies Eyes Shut to Promote Rhabdomere Separation in. PLoS Genet 10(11): e32767. doi:10.1371/journal.pgen.1004795
Kategorie: Research Article
prolekare.web.journal.doi_sk: https://doi.org/10.1371/journal.pgen.1004795

Souhrn

Glycosylation (addition of sugars to proteins and other organic molecules) is important for protein function and animal development. Each form of glycosylation is usually present on multiple proteins. Therefore, a major challenge in understanding the role of sugars in animal development is to identify which protein(s) modified by a specific sugar require the sugar modification for proper functionality. We have previously shown that an enzyme called Rumi adds glucose molecules to an important cell surface receptor called Notch, and that glucose plays a key role in the function of Notch both in fruit flies and in mammals. Using fruit flies, we have now identified a new Rumi target called “Eyes shut”, a secreted protein with a critical role in the optical isolation of neighboring photoreceptors in the fly eye. Our data suggest that glucose molecules on Eyes shut promote its folding and stability in a critical time window during eye development. Mutations in human Eyes shut result in a devastating form of retinal degeneration and loss of vision. Since human Eyes shut is also predicted to harbor glucose molecules, our work provides a framework to explore the role of sugar modifications in the biology of a human disease protein.


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Genetika Reprodukčná medicína

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PLOS Genetics


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