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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Carbonic anhydrase XIV deficiency produces a functional defect in the retinal light response
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Carbonic anhydrase XIV deficiency produces a functional defect in the retinal light response

机译：碳酸酐酶XIV缺乏会在视网膜光反应中产生功能缺陷

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摘要

Members of the carbonic anhydrase (CA) family play an important role in the regulation of pH, CO_2, ion, and water transport. CA IV and CA XIV are membrane-bound isozymes expressed in the eye. CA IV immunostaining is limited to the choriocapillaris overlying the retina, whereas CA XIV is expressed within the retina in Mueller glial cells and retinal pigment epithelium. Here, we have characterized the physiological and morphological phenotype of the CA IV-null, CA XIV-null, and CA IV/CA XIV-double-null mouse retinas. Flash electroretinograms performed at 2, 7, and 10 months of age showed that the rod/cone a-wave, b-wave, and cone b-wave were significantly reduced (26-45%) in the CA XIV-null mice compared with wild-type littermates. Reductions in the dark-adapted response were not progressive between 2 and 10 months, and no differences in retinal morphology were observed between wild-type and CA XIV-null mice. Mueller cells and rod bipolar cells had a normal appearance. Retinas of CA IV-null mice showed no functional or morphological differences compared with normal littermates. However, CA IV/CA XIV double mutants showed a greater deficit in light response than the CA XIV-null retina. Our results indicate that CA XIV, which regulates extracellular pH and pCO_2, plays an important part in producing a normal retinal light response. A larger functional deficit in the CA IV/CA XIV double mutants suggests that CA IV can also contribute to pH regulation, at least in the absence of CA XIV.
机译：碳酸酐酶（CA）家族的成员在调节pH，CO_2，离子和水的运输中起重要作用。 CA IV和CA XIV是在眼睛中表达的膜结合同工酶。 CA IV免疫染色仅限于覆盖视网膜的脉络膜毛细血管，而CA XIV在Mueller神经胶质细胞和视网膜色素上皮的视网膜内表达。在这里，我们已经表征了CA IV-null，CA XIV-null和CA IV / CA XIV-null-null小鼠视网膜的生理和形态表型。在2、7和10个月大时进行的快速视网膜电图检查显示，与未使用CA XIV的小鼠相比，棒/圆锥a波，b波和锥b波显着降低了（26-45％）野生型同窝幼仔。在2到10个月之间，暗适应反应的降低没有进展，在野生型和CA XIV无效小鼠之间未观察到视网膜形态的差异。 Mueller细胞和双极杆状细胞外观正常。与正常同窝仔相比，CA IV空小鼠的视网膜没有功能或形态上的差异。但是，CA IV / CA XIV双突变体显示出比CA XIV空的视网膜更大的光反应缺陷。我们的结果表明，调节细胞外pH和pCO_2的CA XIV在产生正常的视网膜光反应中起重要作用。 CA IV / CA XIV双重突变体中较大的功能缺陷表明，至少在没有CA XIV的情况下，CA IV还可促进pH调节。

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