Eur Respir J. 2012 Jun;39(6):1405-14. Epub 2011 Oct 17.
Targets of anti-endothelial cell antibodies in pulmonary hypertension and scleroderma.
Dib H, Tamby MC, Bussone G, Regent A, Berezné A, Lafine C, Broussard C, Simonneau G, Guillevin L, Witko-Sarsat V, Humbert M, Mouthon L
INSERM U1016, Institut Cochin, Paris, France.
Abstract
Anti-endothelial cell antibodies have been identified in patients with systemic sclerosis with and without pulmonary arterial hypertension and in patients with idiopathic pulmonary arterial hypertension. However, their target antigens remain poorly identified.Sera from 24 patients with systemic sclerosis without pulmonary arterial hypertension, 20 patients with systemic sclerosis with pulmonary arterial hypertension, 30 with idiopathic pulmonary arterial hypertension and 12 healthy controls were collected. Target antigens were identified by 2-D electrophoresis and immunoblotting in protein extracts of human umbilical vein endothelial cells. Targeted antigens were identified by mass spectrometry.Serum IgG from patients with systemic sclerosis with or without pulmonary arterial hypertension and patients with idiopathic pulmonary arterial hypertension specifically recognized 110, 82, and 37 protein spots respectively. Among others, target antigens of AECA included lamin A/C, tubulin beta chain and vinculin. One-dimension immunoblotting experiments confirmed the identification of lamin A/C and tubulin beta chain.In conclusion, our results confirm the presence of AECA in patients with systemic sclerosis with and without pulmonary arterial hypertension and in those with idiopathic pulmonary arterial hypertension and provide evidence for the identification of target antigens of these autoantibodies including lamin A/C and tubulin beta chain.
PMID:22005913