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首页> 外文期刊>Journal of Epithelial Biology & Pharmacology >Activation and Inactivation Mechanisms of Na-HCO3 Cotransporter NBC1
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Activation and Inactivation Mechanisms of Na-HCO3 Cotransporter NBC1

机译：Na-HCO3共转运蛋白NBC1的活化和失活机理

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The electrogenic Na+-HCO3- cotransporter NBC1, also known as NBCe1, is expressed in several tissues such askidney, pancreas, eye, and brain. NBC1 has three variants, which may mediate different physiological functions. Inactivatingmutations in NBC1 result in proximal renal tubular acidosis associated with ocular abnormalities. Functionalanalysis suggests that at least 50% reduction in NBC1 activity is required to induce severe acidemia invariably associatedwith stunted growth. Some mutations in the NBC1 gene may also induce trafficking abnormalities. On the other hand, thevariant-specific N-terminal regions seem to play an essential role in the full activation of NBC1. This review will focus onthe recent progress in physiology and pathophysiology of NBC1.
机译：Na + -HCO3-共转运蛋白NBC1（也称为NBCe1）在多种组织中表达，例如水母，胰腺，眼睛和大脑。 NBC1具有三种变体，可以介导不同的生理功能。 NBC1的失活突变会导致近端肾小管酸中毒，并伴有眼部异常。功能分析表明，NBC1活性至少降低50％才能诱导严重的酸血症，而酸血症总是与发育迟缓相关。 NBC1基因中的某些突变也可能引起运输异常。另一方面，变体特异性N末端区域似乎在NBC1的完全活化中起重要作用。这项审查将侧重于NBC1的生理学和病理生理学的最新进展。

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