Howell D N, Damms T, Burchette J L, Green W R
Department of Pathology, Veterans Affairs Medical Center, Durham, North Carolina, USA.
Invest Ophthalmol Vis Sci. 1997 Aug;38(9):1896-901.
To test the hypothesis that the aberrant, cytokeratin-expressing cells that replace endothelium in the iridocorneal endothelial (ICE) syndrome are of endothelial origin.
Corneas from four patients with Chandler's syndrome and three with essential iris atrophy were examined by two-color immunofluorescence for simultaneous expression of cytokeratins and two markers of endothelial lineage: vimentin and the antigen recognized by the antiendothelial monoclonal antibody 2B4.14.1.
In six corneas, unequivocal endothelial staining for cytokeratins was present; in each of these, cells coexpressing cytokeratins and the two endothelial markers were clearly identifiable. In the remaining cornea, weak cytokeratin staining that colocalized with vimentin was present.
These results lend strong support to the hypothesis that the "epithelial-like" endothelial cells in ICE syndrome are cells of endothelial lineage rather than heterotopia of epithelial cells; these cells probably arise via a metaplastic transformation of preexisting endothelium.
检验虹膜角膜内皮（ICE）综合征中取代内皮的异常表达细胞角蛋白的细胞起源于内皮这一假说。
对4例钱德勒综合征患者和3例原发性虹膜萎缩患者的角膜进行双色免疫荧光检查，以同时检测细胞角蛋白以及内皮谱系的两种标志物波形蛋白和抗内皮单克隆抗体2B4.14.1识别的抗原的表达情况。
在6只角膜中，存在明确的细胞角蛋白内皮染色；在每只角膜中，均可清晰识别出同时表达细胞角蛋白和两种内皮标志物的细胞。在其余1只角膜中，存在与波形蛋白共定位的弱阳性细胞角蛋白染色。
这些结果有力支持了ICE综合征中“上皮样”内皮细胞是内皮谱系细胞而非上皮细胞异位的假说；这些细胞可能是由先前存在的内皮经化生转变而来。
