Fibroblast growth factor receptor deficiency in dystrophic retinal pigmented epithelium.

摘要: The retinal pigmented epithelium (RPE) is known to be site of the primary lesion in inherited dystrophy Royal College Surgeons (RCS) rat, a model for retinitis pigmentosa. Although only functional defect so far detected these cells their failure efficiently phagocytose shed photoreceptor outer segment debris, actual cause cell death still unknown. Recently possibility “trophic factors” important survival produced by normal RPE but not dystrophic has been suggested. Hence we decided investigate presence and abundance two candidate diffusible factors, acidic basic fibroblast growth factors (aFGF bFGF, respectively), as well high affinity surface receptors (FGF-R). mRNA was isolated from cultures purified analyzed PCR amplification using specific oligonucleotide primers aFGF bFGF: size amplified fragments similar both types. Also, protein, immunocytochemistry antisera, appeared present approximately equal amounts distributed pattern. However, scatchard analysis radio-labelled bFGF binding rat revealed that possess 29% number compared congenic cells. Furthermore, level expression FGF-R2 mRNA, FGF-R1, significantly different. Other parameters measured (receptor affinity, profile ligand internalization degradation, receptor molecular weight mitogenic activity) did show any significant differences between RPE. precise role FGF-R deficiency etiology disease hence remains determined, it indicates importance trophic functioning retina. © 1993 Wiley-Liss, Inc.