Hamed Jafar-Nejad
Department of Molecular and Human Genetics,
Program in Developmental Biology
Baylor College of Medicine
Hamed Jafar-Nejad is an Associate Professor in the Department of Molecular & Human Genetics and the Program in Developmental Biology at Baylor College of Medicine. His group is interested in the roles of glycosylation and deglycosylation in the regulation of animal development and human disease pathogenesis.
He received his MD degree from Tehran University of Medical Sciences and learned basic molecular biology techniques in a research institute in Iran. He spent one year in the Neuroscience Research Institute at the University of Ottawa, studying the transcriptional regulation of a serotonin receptor implicated in mood disorders with Dr. Paul Albert. He then moved to Houston and started his postdoctoral training in the area of Notch signaling and Drosophila neurogenesis with Dr. Hugo Bellen at Baylor College of Medicine/HHMI. In December 2006, he joined the faculty at the University of Texas Health Science Center at Houston, focusing on a glycosyltransferase called Rumi which he had identified in Drosophila as a key regulator of the Notch signaling pathway.
In 2012, he was recruited back to the Department of Molecular & Human Genetics at Baylor, where his group continues their work on the role of glycosylation in animal development and human disease. Hamed’s lab has established a mouse model for a rare disease called Alagille syndrome and has identified Rumi (Poglut1) as a dominant genetic suppressor of the Alagille biliary phenotypes in the mouse. The Jafar-Nejad group is also using Drosophila and mammalian cell culture assays to understand the role of a deglycosylation enzyme called N-glycanase 1 (NGLY1) in animal development and BMP signaling, in hopes of shedding light on the pathophysiology of NGLY1 deficiency in human patients and identifying drug targets for this disease.
Mapping Drosophila mutations with molecularly defined P element insertions.
Proceedings of the National Academy of Sciences of the United States of America Sep, 2003 | Pubmed ID: 12960394
Senseless acts as a binary switch during sensory organ precursor selection.
Genes & development Dec, 2003 | Pubmed ID: 14665671
Gfi/Pag-3/senseless zinc finger proteins: a unifying theme?
Molecular and cellular biology Oct, 2004 | Pubmed ID: 15456856
The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins.
Cell Aug, 2005 | Pubmed ID: 16122429
Sec15, a component of the exocyst, promotes notch signaling during the asymmetric division of Drosophila sensory organ precursors.
Developmental cell Sep, 2005 | Pubmed ID: 16137928
The tumour-suppressor genes NF2/Merlin and Expanded act through Hippo signalling to regulate cell proliferation and apoptosis.
Nature cell biology Jan, 2006 | Pubmed ID: 16341207
Senseless and Daughterless confer neuronal identity to epithelial cells in the Drosophila wing margin.
Development (Cambridge, England) May, 2006 | Pubmed ID: 16554363
Senseless physically interacts with proneural proteins and functions as a transcriptional co-activator.
Development (Cambridge, England) May, 2006 | Pubmed ID: 16624856
Rumi is a CAP10 domain glycosyltransferase that modifies Notch and is required for Notch signaling.
Cell Jan, 2008 | Pubmed ID: 18243100
Novel function of the class I bHLH protein Daughterless in the negative regulation of proneural gene expression in the Drosophila eye.
EMBO reports Nov, 2008 | Pubmed ID: 18758436
Role of glycans and glycosyltransferases in the regulation of Notch signaling.
Glycobiology Aug, 2010 | Pubmed ID: 20368670
Regulation of mammalian Notch signaling and embryonic development by the protein O-glucosyltransferase Rumi.
Development (Cambridge, England) May, 2011 | Pubmed ID: 21490058
Multiple O-glucosylation sites on Notch function as a buffer against temperature-dependent loss of signaling.
Development (Cambridge, England) Aug, 2011 | Pubmed ID: 21771811
Negative regulation of notch signaling by xylose.
PLoS genetics Jun, 2013 | Pubmed ID: 23754965
Structure-function analysis of Drosophila Notch using genomic rescue transgenes.
Methods in molecular biology (Clifton, N.J.) , 2014 | Pubmed ID: 25053479
Fringe proteins modulate Notch-ligand cis and trans interactions to specify signaling states.
eLife Sep, 2014 | Pubmed ID: 25255098
The protein O-glucosyltransferase Rumi modifies eyes shut to promote rhabdomere separation in Drosophila.
PLoS genetics Nov, 2014 | Pubmed ID: 25412384
A congenital disorder of deglycosylation: Biochemical characterization of N-glycanase 1 deficiency in patient fibroblasts.
Glycobiology Aug, 2015 | Pubmed ID: 25900930
The multiple roles of epidermal growth factor repeat O-glycans in animal development.
Glycobiology Oct, 2015 | Pubmed ID: 26175457
Jagged1 heterozygosity in mice results in a congenital cholangiopathy which is reversed by concomitant deletion of one copy of Poglut1 (Rumi).
Hepatology (Baltimore, Md.) Feb, 2016 | Pubmed ID: 26235536
Mapping Sites of O-Glycosylation and Fringe Elongation on Drosophila Notch.
The Journal of biological chemistry 07, 2016 | Pubmed ID: 27268051
A POGLUT1 mutation causes a muscular dystrophy with reduced Notch signaling and satellite cell loss.
EMBO molecular medicine 11, 2016 | Pubmed ID: 27807076
Xylosylation of the Notch receptor preserves the balance between its activation by trans-Delta and inhibition by cis-ligands in Drosophila.
PLoS genetics Apr, 2017 | Pubmed ID: 28394891
Tissue-specific regulation of BMP signaling by Drosophila N-glycanase 1.
eLife Aug, 2017 | Pubmed ID: 28826503
Generation of an induced pluripotent stem cell line (CSCRMi001-A) from a patient with a new type of limb-girdle muscular dystrophy (LGMD) due to a missense mutation in POGLUT1 (Rumi).
Stem cell research Oct, 2017 | Pubmed ID: 29034878
A New Model of Alagille Syndrome With Broad Phenotypic Representation.
Gastroenterology 03, 2018 | Pubmed ID: 29425927
Sensitized genetic backgrounds reveal differential roles for EGF repeat xylosyltransferases in Drosophila Notch signaling.
Glycobiology Aug, 2018 | Pubmed ID: 30169771
Unbiased glycomics: a powerful tool in rare disease diagnosis and research.
Translational research : the journal of laboratory and clinical medicine 04, 2019 | Pubmed ID: 30528322
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