A case of primary immunodeficiency due to a defect of the major histocompatibility gene complex class I processing and presentation pathway

H Teisserenc, W Schmitt, N Blake, R Dunbar… - Immunology letters, 1997 - Elsevier
Introduction: We report a case of primary immunodeficiency due to a defect of the TAP
transporter, an heterodimeric complex which controls the expression of HLA class I molecule
by delivering peptides from the cytosol into the lumen of the endoplasmic reticulum. Since
childhood, the 36 year old female suffered from recurrent sinusitis/bronchitis. She later
developed bronchiectasis and destructive nasal epitheloid granulomata in conjunction with
a generalized vasculitic syndrome that did not improve upon immunosuppression and …