Rachel Rivka A, Mason Carol A, Beermann Friedrich
Center for Neurobiology and Behaviour, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
Pigment Cell Res. 2002 Aug;15(4):273-81. doi: 10.1034/j.1600-0749.2002.02019.x.
To study the relationship among tyrosinase activity, melanin production, and the routing of retinal ganglion cell (RGC) axons at the optic chiasm, we analysed mice with varying doses of the tyrosinase gene. These include the dark-eyed albino (Tyrc44H), a radiation-induced hypomorphic allele of tyrosinase; and transgenic mice carrying 1 or 2 alleles of a tyrosinase minigene on both wild-type (Tyr+) and albino (Tyrc) backgrounds. Melanization of the retinal pigment epithelium (RPE) occurred gradually even at <2% wild-type tyrosinase activity and was sensitive to tyrosinase activity up to <35% of wild-type levels, beyond which melanin synthesis appeared to be saturated. Overexpression of tyrosinase led to tyrosinase activity above wild type level, but did not increase melanin production. Although a loss of melanin because of a mutation in tyrosinase is associated with a decrease in the number of uncrossed fibers, elevating tyrosinase levels does not appear to cause an increase in the size of the uncrossed retinal projection. Our results suggest that replacing less than 35% of wild-type tyrosinase activity is sufficient to restore normal pigmentation of the RPE, and potentially, to allay visual defects.
为了研究酪氨酸酶活性、黑色素生成与视网膜神经节细胞(RGC)轴突在视交叉处的投射路径之间的关系,我们分析了具有不同剂量酪氨酸酶基因的小鼠。这些小鼠包括黑眼白化小鼠(Tyrc44H),它是一种经辐射诱导产生的酪氨酸酶低表达等位基因;以及在野生型(Tyr+)和白化(Tyrc)背景下携带1个或2个酪氨酸酶小基因等位基因的转基因小鼠。即使在野生型酪氨酸酶活性低于2%时,视网膜色素上皮(RPE)的黑色素沉着也会逐渐发生,并且在野生型水平低于35%时对酪氨酸酶活性敏感,超过该水平后黑色素合成似乎达到饱和。酪氨酸酶的过表达导致其活性高于野生型水平,但并未增加黑色素生成。虽然由于酪氨酸酶突变导致的黑色素缺失与未交叉纤维数量的减少有关,但提高酪氨酸酶水平似乎并不会导致未交叉视网膜投射的增大。我们的结果表明,替代低于35%的野生型酪氨酸酶活性足以恢复RPE的正常色素沉着,并有可能缓解视觉缺陷。