1. Alpha-mannosidase-II deficiency results in dyserythropoiesis andunveils an alternate pathway in oligosaccharide biosynthesis.Chui D, Oh-Eda M, Liao YF, Panneerselvam K, Lal A, Marek KW, Freeze HH,Moremen KW, Fukuda MN, Marth JD.Cell. 1997 Jul 11;90(1):157-67.2. Genetic remodeling of protein glycosylation in vivo inducesautoimmune disease.Chui D, Sellakumar G, Green R, Sutton-Smith M, McQuistan T, Marek K, Morris H,Dell A, Marth JD.Proc Natl Acad Sci U S A. 2001 Jan 30;98(3):1142-7.3. Mammalian N-glycan branching protects against innate immune self-recognition and inflammation in autoimmune disease pathogenesis.Green RS, Stone EL, Tenno M, Lehtonen E, Farquhar MG, Marth JD.Immunity. 2007 Aug;27(2):308-20.Biomedical GlycobiologyFrontier of N-glycan studyFunction of α-Mannosidase II in N-glycan BiosynthesisCell. 1997Generation of α-Mannosidase II Knockout MiceCell. 1997α-Mannosidase II Enzyme ActivityCell. 1997The α-Mannosidase II Knockout Mice Develop Dyserythropoietic AnemiaCell. 1997Anisocytosis:a patient's red blood cells are ofunequal sizeLittle i antigen: poly-N-acetyllactosamineLarge I antigen: poly-N-acetyllactosamine + β1-6GlcNAc branchThe α-Mannosidase II-/- Erythrocytes Lack Complex N-glycansCell. 1997LectinE-PHAThe α-Mannosidase II Deficiency Reduces Complex-, Increases Hybrid N-glycansPNAS. 2001The α-Mannosidase II Mice Develop GlomerulonephritisPNAS. 2001KidneyLiverThe Hematopoietic and Immune Parameters in the α-Mannosidase II MicePNAS. 2001Presence of Autoantibodies in the α -Mannosidase II MicePNAS. 2001Nonhematopoietic Origin of Autoimmune Disease in αM-II DeficiencyImmunity. 2007Disease in the Absence of Adaptive Immune System and IgG TreatmentImmunity. 2007RAG-1 is essential to the generation of matureB and T lymphocytes, two cell types that arecrucial components of the adaptive immunesystem.Pathogenesis in the Absence of Complement C3Immunity. 2007C3 plays a central role in the complement system andcontributes to innate immunity(macrophage)Innate Immune Activation and Kidney inflammationImmunity. 2007Immunity. 2007Mesangial Cell Activation and ApoptosisMesangial Cell Activation and ApoptosisImmunity. 2007Mesangial Cell Activation and ApoptosisImmunity. 2007Immunity Preview. 2007Self Glycan Triggers an Innate Immune Response in Mice Lacking α-mannosidase IIHave a Fun Summer !Evaluation
View Full Document
Unlocking...