| 特性など: |
網膜障害。ヒト網膜色素変性症のモデル動物。
原因遺伝子rdy （retinal dystrophy）は、チロシンキナーゼ受容体をコードするMertk遺伝子の欠失変異であることが判明した（D'Cruz, 2000）。網膜障害は3週齢より進行性に起こる（Bourne, 1938）。通常、光受容体細胞の一つである杆状体の外節は、色素上皮細胞による貪食と再生を繰り返しているが、RCSでは色素上皮細胞の貪食機能が障害されている。そのため未分解の杆状体外節断片が蓄積し、杆状体および錐状体が細胞死を起こしている（Dowling, 1962; Tuo, 1994）。光受容体細胞の進行性変性に対する治療実験が多数報告されている（Faktorovich, 1990; Zhang, 2009; Thumann, 2009; Lu, 2010）。ピンクアイダイリューションOca2（別名P）に欠失変異を持ち、薄桃色の眼を示す（Kuramoto, 2005）。（Oct 1, 2010） |
| 参考文献: |
1. Bourne MC, Campbell DA, Pyke M. Cataract associated with an hereditary retinal lesion in rats. Br J Ophthalmol. 1938 Oct;22(10):608-613.
2. Bourne MC, Campbell DA, Tansley K. Hereditary degeneration of the rat retina. Br J Ophthalmol. 1938 Oct;22(10):613-623.
3. Dowling JE, Sidman RL. Inherited retinal dystrophy in the rat. J Cell Biol. 1962 Jul;14:73-109.
4. LaVail MM, Sidman RL, Gerhardt CO. Congenic strains of RCS rats with inherited retinal dystrophy. J Hered. 1975 Jul-Aug;66(4):242-244.
5. Edwards RB, Szamier RB. Defective phagocytosis of isolated rod outer segments by RCS rat retinal pigment epithelium in culture. Science. 1977 Sep 2;197(4307):1001-1003.
6. LaVail MM. Photoreceptor characteristics in congenic strains of RCS rats. Invest Ophthalmol Vis Sci. 1981 May;20(5):671-675.
7. Faktorovich EG, Steinberg RH, Yasumura D, Matthes MT, LaVail MM. Photoreceptor degeneration in inherited retinal dystrophy delayed by basic fibroblast growth factor. Nature. 1990 Sep 6;347(6288):83-86.
8. Tso MO, Zhang C, Abler AS, Chang CJ, Wong F, Chang GQ, Lam TT. Apoptosis leads to photoreceptor degeneration in inherited retinal dystrophy of RCS rats. Invest Ophthalmol Vis Sci. 1994 May;35(6):2693-9.
9. D'Cruz PM, Yasumura D, Weir J, Matthes MT, Abderrahim H, LaVail MM, Vollrath D. Links Mutation of the receptor tyrosine kinase gene Mertk in the retinal dystrophic RCS rat. Hum Mol Genet. 2000 Mar 1;9(4):645-651.
10. Kuramoto T, Gohma H, Kimura K, Wedekind D, Hedrich HJ, Serikawa T. The rat pink-eyed dilution (p) mutation: an identical intragenic deletion in pink-eye dilute-coat strains and several Wistar-derived albino strains. Mamm Genome. 2005 Sep;16(9):712-719.
11. Zhang M, Mo X, Fang Y, Guo W, Wu J, Zhang S, Huang Q. Rescue of photoreceptors by BDNF gene transfer using in vivo electroporation in the RCS rat of retinitis pigmentosa. Curr Eye Res. 2009 Sep;34(9):791-799.
12. Thumann G, Salz AK, Walter P, Johnen S. Preservation of photoreceptors in dystrophic RCS rats following allo- and xenotransplantation of IPE cells. Graefes Arch Clin Exp Ophthalmol. 2009 Mar;247(3):363-9.
13. Lu B, Wang S, Girman S, McGill T, Ragaglia V, Lund R. Human adult bone marrow-derived somatic cells rescue vision in a rodent model of retinal degeneration. Exp Eye Res. 2010 Sep;91(3):449-455.
14. Tsuji N, Ozaki K, Narama I, Matsuura T. Inferior ectopic pupil and typical ocular coloboma in RCS rats. Comp Med. 2011 Aug;61(4):378-384.
15. Liu K, Wang Y, Yin Z, Weng C, Zeng Y. Changes in glutamate homeostasis cause retinal degeneration in Royal College of Surgeons rats. Int J Mol Med. 2013 May;31(5):1075-1080.
16. Leow SN, Luu CD, Hairul Nizam MH, Mok PL, Ruhaslizan R, Wong HS, Wan Abdul Halim WH, Ng MH, Ruszymah BH, Chowdhury SR, Bastion ML, Then KY. Safety and Efficacy of Human Wharton's Jelly-Derived Mesenchymal Stem Cells Therapy for Retinal Degeneration. PLoS One. 2015 Jun 24;10(6):e0128973
16. Terayama Y, matsuura T, Ozaki K. Malignant mast cell tumor of the thymus in a Royal College of Surgeons (RCS) rat. J Toxicol Pathol. Article ID: 2016-0044
|