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类似先天性静止性夜盲症视觉电生理异常大鼠1例 被引量:4

A rat with retinal hereditary disease similar to congenital stationary night blindness patient's ERG
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摘要 目的 观察正常 SD大鼠和一例患遗传性视网膜疾病大鼠的视觉电生理学特点 .方法 按国际临床视觉电生理学会标准化方案 ,采用 REPIport系统和自制的氯化银角膜电极、不锈钢针状电极对成年雄性 SD大鼠进行视网膜电图暗适应 (ERG)、振荡电位 (OPs波 )和明适应 ERG进行观察 .结果 正常大鼠 ERG特性与人类相似 ,一只大鼠视觉电生理明显异常 ,ERG呈负波型 ,表现为暗适应 ERG b波基本消失 ,暗适应 ERG a波幅值明显降低 ,明适应 ERG a,b波幅值降低 ,峰时延长 ,OPs波幅值基本消失 .该例大鼠的视觉电生理学特点与人类先天性静止性夜盲症患者视觉电生理特点相似 ,而与视网膜色素变性病例不同 .结论 发现一例自然发生的基因突变大鼠 。 AIM To compare the characteristic of the visual electrophysiology between normal SD rats and a case of abnormal rat with retinal hereditary disease. METHODS According to the International Clinic Visual ElectrophysiologicalStandard Program, 22 adult male SD rats were tested to observe the scotopic retinoelectrogram (Scot. ERG), photopic retinoelectrogram (Phot. ERG) and oscillatory potentials (OPs) with RETIport system, Ag AgCl corneal contact electrode and stainless steel needle electrode. RESULTS The ERGs and OPs waves of the normal 21 rats were similar to that of human. However, one of the case (both eyes) was abnormal. ERGs and OPs. The Scot. ERGs showed a negative wave, in which the b wave almost disappeared and the amplitude of a wave obviously descended. The amplitude of a, b wave of Phot. ERGs declined, the letancy delayed. The amplitude of the OPs almost disappeared. The electrophysiology character of the rat was similar to the human Congenital Stationary Night Blindness, but very different from retinal pigmentosa. CONCLUSION One case of congenital gene mutation rat are found. The clinical visual electrophysiology test shows that the rat had inherited retinal degeneration disease.
出处 《第四军医大学学报》 北大核心 2002年第1期5-7,共3页 Journal of the Fourth Military Medical University
关键词 遗传性视网膜疾病 视网膜电图 先天性静止性夜盲症 动物模型 retinal hereditary disease rat electroretinogram congenital stationary night blindness animal model
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参考文献7

  • 1Bayer AU, Cook P, Brodie SE, Maag KP, Mittag T. Evaluation of different reco rding parameters to establish a standard for flash electroretinography in rodent s [J]. Vision Res, 2001;41(17):2173-2185.
  • 2Bech-Hansen NT, Naylor MJ, Maybaum TA, Sparkes RL, Koop B, Birch DG, Bergen A A, Prinsen CF, Polomeno RC, Gal A, Drack AV, Musarella MA, Jacobson SG, Young RS, Weleber RG. Mutations in NYX, encoding the leucine-rich proteoglycan nyctalop i n, cause X-linked complete congenital stationary night blindness [J]. Nat G enet, 2000; 26(3):319-323.
  • 3Bech-Hansen NT, Naylor MJ, Maybaum TA, Pearce WG, Koop B, Fishman G A, Mets M, Musarella MA, Boycott KM. Loss-of-function mutations in a calcium-channel a lpha1-subunit gene in Xp11.23 cause incomplete X-linked congenital stationary night blindness [J]. Nat Genet, 1998; 19(3): 264-267.
  • 4Strom TM, Nyakatura G, Apfelstedt-Sylla E, Hellebrand H, Lorenz B, Weber BH, Wutz K, Gutwillinger N, Ruther K, Drescher B, Sauer C, Zrenner E, Meitinger T, R osenthal A, Meindl A. An L-type calcium-channel gene mutated in incomplete X-linked congenital stationary night blindness [J]. Nat Genet, 1998; 19(3): 260-263.
  • 5Pusch CM, Zeitz C, Brandau O, Pesch K, Achatz H, Feil S, Scharfe C, Maurer J, Jacobi FK, Pinckers A, Andreasson S, Hardcastle A, Wissinger B, Berger W, Meind l A. The complete form of X-linked congenital stationary night blindness is ca used by mutations in a gene encoding a leucine-rich repeat protein [J]. Nat Genet, 2000;26(3):324-327.
  • 6Flannery JG. Transgenic animal models for the study of inherited ret inal dystrophies [J]. ILAR J, 1999; 40(2): 51-58.
  • 7Pardue MT, McCall MA, LaVail MM, Gregg RG, Peachey NS. A natural ly occur ring mouse model of X-linked congenital stationary night blindness [J]. Inv est Ophthalmol Vis Sci, 1998; 39(12):2443-2449.

同被引文献53

  • 1赵朝霞,董应丽,李蕴随,尹卫靖,郭希让.视网膜色素变性和先天性静止性夜盲的mERG检查[J].眼科研究,2003,21(3):282-285. 被引量:2
  • 2陈华,李春海,贺苏兰,乔彦芝,马天富.性别因素对大鼠部分血液学、血液生化指标和脏器系数的影响[J].实验动物科学与管理,1996,13(1):21-23. 被引量:33
  • 3Boycott KM, Pearce WG, Musarella MA, et al. Evidence for genetic heterogeneity in X-linked congenital stationary night blindness. Am J Hum Genet, 1998,62:865-875.
  • 4Bech-Hansen NT, Naylor MJ, Maybaum TA, et al. Loss-of-functionmutations in a calcium-channel alpha-l-subunit gene in Xpll. 23 cause incomplete X-linked congenital stationary night blindness. Nat Genet, 1998,19 : 264-267.
  • 5Strom TM, Nyakatura G, Apfelstedt-SyllaE, et al. An L-type calcium-channel gene mutated in incomplete X-linked congenital stationary night blindness. Nat Genet, 1998,19 : 260-263.
  • 6Boycott KM, Pearce WG, Bech-Hansen NT. Clinical variability among patients with incomplete X-linked congenital stationary night blindness and a founder mutation in CACNA1F. Can J Ophthalmol,2000,35:204-213.
  • 7Yong RS, Chaparro A, Price J, et al. Oscillatory potentials of Xlinked carriers of congenital stationary night blindness ( I-CSNB ).Invest Ophthalmol Vis Sci, 1989, 30: 806-812.
  • 8Rigaudiere F, Roux C, Lachapelle p, et al. ERGs in female carriers of incomplete congenital stationary night blindness. Doc Ophthalmol,2003, 107: 203-212.
  • 9Sieving PA. Photopic ON- and Off-pathway abnormalities in retinal dystrophies. Trans Am Ophthahnol, 1993, 91.701-773.
  • 10Langrova H,Gamer D,Friedberg C,et al.Abnormalities of the long flash ERG in congetnital stationary aight blindness of the SchubertBomschein. Vis Res, 2002, 42 : 1475-1483.

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