| NBRP Rat No | 0011 |
|---|---|
| 品系名稱 | ACI/NKyo |
| 俗名 | ACI/NKyo |
| 毛色 | tan hooded (a,B,C,h,p,R) |
| 近交系世代 | F18+29 (March 2012) |
| Genetic Status | Inbred, Spont. Mutant |
| 應用 | 眼科(Ophthalmology) |
| Gene Affected | Mertk: c-mer proto-oncogene tyrosine kinase |
| 來源 | 在1938年,在一隻帶有粉紅色眼睛的有色大鼠中,發現遺傳性視網膜異常的個體(Bourne, 1983)。於1998年從京都大學醫學部眼科學教室搬遷至京都大學醫學研究科附屬動物實驗設施。RCS是Royal College of Surgeons的縮寫。(2010年10月1日) |
| 特徵 | 視網膜障礙。人類視網膜色素病變(retinitis pigmentosa)的模型動物。 其致病基因rdy (視網膜失養症, retinal dystrophy)是由於encode 酪氨酸激酶受體(tyrosine kinase receptor)的Mertk基因的刪除突變所致(D'Cruz, 2000)。視網膜障礙從3週齡起開始逐步發展(Bourne, 1938)。通常,感光細胞之一的視桿細胞的外節(outer segment)會被色素上皮細胞吞噬和再生。但在RCS大鼠,色素上皮細胞的吞噬功能受到損害。 因此,未被分解的視桿細胞外節片段會堆積,視桿細胞和視錐細胞經歷細胞凋亡(Dowling, 1962; Tuo, 1994)。已有多項針對光受體細胞進行性退化的治療實驗被報道(Faktorovich, 1990; Zhang, 2009; Thumann, 2009; Lu, 2010)。 粉紅眼稀釋:此突變在Oca2 (又名P)有刪除突變,並呈現淺粉紅色眼睛。 |
| 繁殖狀態 | 兄妹交配。良好的繁殖性。 |
Reference 參考文獻
- Bourne MC, Campbell DA, Pyke M. Cataract associated with an hereditary retinal lesion in rats. Br J Ophthalmol. 1938 Oct;22(10):608-613.
- Bourne MC, Campbell DA, Tansley K. Hereditary degeneration of the rat retina. Br J Ophthalmol. 1938 Oct;22(10):613-623.
- Dowling JE, Sidman RL. Inherited retinal dystrophy in the rat. J Cell Biol. 1962 Jul;14:73-109.
- LaVail MM, Sidman RL, Gerhardt CO. Congenic strains of RCS rats with inherited retinal dystrophy. J Hered. 1975 Jul-Aug;66(4):242-244.
- 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.
- LaVail MM. Photoreceptor characteristics in congenic strains of RCS rats. Invest Ophthalmol Vis Sci. 1981 May;20(5):671-675.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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[17] 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.





