Invest Ophthalmol Vis Sci. 2009 Jun 10; Byström B, Carracedo S, Behndig AB, Gullberg D, Pedrosa-Domellof FPurpose: To examine the distribution of integrin alpha11 chain in the human cornea during fetal development and in normal and diseased adult human corneas. Methods: Six fetal corneas, 10-20 weeks of gestation (wg), and 18 adult corneas including 3 normal, 7 with keratoconus, 5 with pseudophakic bullous keratopathy (PBK), two with Fuchs' corneal dystrophy and one with a scar after previous deep lamellar keratoplasty (DLKP) were processed for immunohistochemistry with specific antibodies against integrin alpha11 chain, collagen I, IV and V and alpha-smooth muscle actin (alpha-SMA). The cellular source of alpha11 integrin chain was further investigated in cell cultures. Results: At 10-17 wg alpha11 integrin chain was predominantly present in the anterior corneal stroma. At 20 wg, in normal adult corneas and in Fuchs' dystrophy corneas there was weak staining in the stroma. The PBK corneas showed variable and weak staining, generally accentuated in the posterior stroma near the Descemet's membrane. In contrast, the anterior portion of the stroma in the keratoconus corneas was strongly stained in an irregular streaky pattern. Human corneal fibroblasts/myofibroblasts produced alpha11 integrin chain in culture. Cultures treated with TGF-beta showed higher content of both alpha-SMA and alpha11 integrin chain. Conclusions: The presence of alpha11 integrin chain during early corneal development and the enhanced expression in scarred keratoconus corneas indicates that this integrin chain likely plays an important role in collagen deposition during corneal development and in keratoconus with a scarring component and compromised basement membrane integrity.