L. Takacs et al., TRANSFORMING-GROWTH-FACTOR-BETA INDUCED PROTEIN, BETA-IG-H3, IS PRESENT IN DEGRADED FORM AND ALTERED LOCALIZATION IN LATTICE CORNEAL-DYSTROPHY TYPE-I, Experimental Eye Research, 66(6), 1998, pp. 739-745
Lattice corneal dystrophy type I (LCDI) is an inherited autosomal domi
nant local amyloidosis, restricted to the corneal stroma. Comparison o
f electrophoretic profiles of normal and dystrophic corneas revealed a
42 kD protein, which was present only in dystrophic corneas. The N-te
rminal sequence of this protein showed identity to transforming growth
factor-beta induced gene product (beta IG-H3). A polyclonal antiserum
was raised in chicken against a synthetic peptide identical to the N-
terminal portion of beta IG-H3. On immunoblots, the antiserum stained
the 42 kD band, and also a 68 kD band corresponding to the reported mo
lecular weight of the intact beta IG-H3. In normal corneas, only the 6
8 kD band was present. Immunohistologically, the antiserum stained cor
neal subepithelial regions, including subepithelial deposits, in dystr
ophic corneas. In normal corneas, the staining was observed only in th
e epithelium. These results may reflect the role of beta IG-H3 in extr
acellular matrix construction and/or amyloid formation. (C) 1998 Acade
mic Press.