【摘 要】
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Post-transitional modification (PTM) is a ubiquitous cellular process.In recent decades an exponential understanding of defects in glycan assembly and processing (N-glycan/O-glyan or combined), has le
【机 构】
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Department of Metabolic Medicine The Royal Children's Hospital School of Medicine University of Que
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Post-transitional modification (PTM) is a ubiquitous cellular process.In recent decades an exponential understanding of defects in glycan assembly and processing (N-glycan/O-glyan or combined), has lead the way in identifying congenital defects of a specific PTM process ie congenital disorders of glycosylation.Secondary modifications in glycan structures in other metabolic disorders have been associated with modifications in disease phenotypic expression and dysregulated unfolded protein responses eg Galactosaemia and select neurodegenerative diseases.However: glycosylation is but one of many PTM process.SUMOylation is one such PTM in which potential exists for the discovery of novel syndromes and treatment paradigms.PTM by small ubiquitin-like modifier (SUMO) controls diverse cellular processes including transcriptional regulation, cell cycle progression, DNA repair, nuclear transport and signal transduction pathways.SUMOylation is a highly dynamic and reversible process.Opportunity exists for improved understanding of how SUMOylation modifies disease common expression, how primary SUMOylation defects eerate novel multi-system genetic syndromes, manipulation of the SUMOylation process in cancer drag development.
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