2017 Glycobiology Significant Achievement AwardThe Glycobiology Significant Achievement Award is given annually by Oxford University Press (publisher of Glycobiology) to honor a new or mid-career scientist that has made a key discovery during their early careers with the potential to have a substantial impact on the glycoscience community. This year, Oxford is delighted to present the Glycobiology Significant Achievement Award to Dr. Hamed Jafar-Nejad, Associate Professor in the Department of Molecular and Human Genetics at Baylor College of Medicine. The award will be given to Dr. Jafar-Nejad at the Society for Glycobiology Annual meeting this November in Portland, Oregon. Dr. Jafar-Nejad has made significant contributions in multiple areas, including our understanding of O-glucosylation of the Notch receptor and of molecular mechanisms affected in patients with NGLY1 deficiency. Regarding O-glucosylation of Notch, Dr. Jafar-Nejad demonstrated that the enzyme which adds O-glucose to EGF repeats in the extracellular domain of the Notch receptor (Rumi in flies, POGLUT1 in mice) is essential for development in both flies and mice, and that addition of O-glucose is not only essential for Notch activity, but for proper function of other proteins as well (e.g. a fly protein called Eyes shut, mutations in whose human homolog cause autosomal recessive retinitis pigmentosa). His lab has generated a mouse model of the human disease Alagille syndrome, an autosomal dominant disorder caused by mutations in the Notch ligand Jagged1, and shown that reducing the gene dosage of Poglut1 can rescue some phenotypes, suggesting a role for O-glucosylation in this disease. His lab has gone on to demonstrate that elongation of O-glucose residues with xylose on Notch is inhibitory, raising the possibility that Notch activity can be increased or decreased by alterations in the O-glucose glycan structures. Regarding NGLY1, Dr. Jafar-Nejad’s group has studied Ngly1-mutant flies and has recently reported that Ngly1 is required in the fly mesoderm to regulate BMP signaling. They are using these mutants to identify relevant targets of NGLY1 that could lead to therapies for this disease. Thus, Dr. Jafar-Nejad has made several notable contributions that have significantly impacted the glycoscience community, and have the potential to benefit families suffering from Alagille Syndrome and NGLY1 deficiency. For these reasons, Oxford is proud to honor him with this year’s Glycobiology Significant Achievement Award. |