O. Strauss et al., The Royal College of Surgeons rat: An animal model for inherited retinal degeneration with a still unknown genetic defect, ACT ANATOM, 162(2-3), 1998, pp. 101-111
The Royal College of Surgeons (RCS) rat is the first known animal with inhe
rited retinal degeneration. Despite the fact that the genetic defect is not
known, the RCS rat is widely used for research in hereditary retinal dystr
ophies, This review tries to summarize observations which have been made in
the RCS rat and to make an attempt to formulate candidate genes which may
the cause for the retinal degeneration in this rat strain, The genetic defe
ct in RCS rats causes the inability of the retinal pigment epithelium (RPE)
to phagocytose shed photoreceptor outer segments. In normal rats or humans
, this circadian process is regulated by both the cyclic adenosine monophos
phate (cAMP) and the calcium/ inositol phosphate systems. The calcium/inosi
tol phosphate system seems to be linked to the phagocytosis receptors which
recognize photoreceptor outer membranes to initialize phagocytosis, The cA
MP system appeared as modulator of the regulation of phagocytosis. An incre
ase in the intracellular cAMP concentration is an 'off' signal for phagocyt
osis. In RPE cells from RCS rats many observations have been made which ind
icate a changed second messenger metabolism concerning both the cAMP and th
e calcium/inositol phosphate systems, The genetic defect seems to concern a
protein which is involved in the initialization of a second messenger path
way. We conclude that the genes coding for the phagocytosis receptor or for
proteins which are linked to receptors (for example G proteins) are good c
andidates for defective genes in RCS rats.