Department of Pharmacology, School of Medicine, Case Western Reserve University, Cleveland, OH, USA.
Department of Pharmacology, School of Medicine, Case Western Reserve University, Cleveland, OH, USA.
J Lipid Res. 2022 Jun;63(6):100215. doi: 10.1016/j.jlr.2022.100215. Epub 2022 Apr 19.
Vitamin A is distributed within the body to support chromophore synthesis in the eyes and retinoid signaling in most other tissues. Two pathways exist for the delivery of vitamin A: the extrinsic pathway transports dietary vitamin A in lipoproteins from intestinal enterocytes to tissues, while the intrinsic pathway distributes vitamin A from hepatic stores bound to serum retinol binding protein (RBP). Previously, the intestine-specific homeodomain transcription factor (ISX) and the RBP receptor STRA6 were identified as gatekeepers of these pathways; however, it is not clear how mutations in the corresponding genes affect retinoid homeostasis. Here, we used a genetic dissection approach in mice to examine the contributions of these proteins in select tissues. We observed that ISX deficiency increased utilization of both preformed and provitamin A. We found that increased storage of retinoids in peripheral tissues of ISX-deficient mice was dependent on STRA6 and induced by retinoid signaling. In addition, double-mutant mice exhibited a partial rescue of the Stra6 mutant ocular phenotype. This rescue came at the expense of a massive accumulation of vitamin A in other tissues, demonstrating that vitamin A is randomly distributed when present in excessive amounts. Remarkably, provitamin A supplementation of mutant mice induced the expression of the RBP receptor 2 in the liver and was accompanied by increased hepatic retinyl ester stores. Taken together, these findings indicate dynamic crosstalk between the delivery pathways for this essential nutrient and suggest that hepatic reuptake of vitamin A takes place when excessive amounts circulate in the blood.
维生素 A 在体内分布,以支持眼睛中的发色团合成和大多数其他组织中的类视黄醇信号传导。有两种途径可以输送维生素 A:外源性途径将脂蛋白中的膳食维生素 A 从肠上皮细胞转运到组织,而内源性途径则将与血清视黄醇结合蛋白(RBP)结合的肝储存中的维生素 A 分配。以前,肠特异性同源域转录因子(ISX)和 RBP 受体 STRA6 被鉴定为这些途径的守门员;然而,尚不清楚相应基因的突变如何影响类视黄醇的动态平衡。在这里,我们使用小鼠的遗传剖析方法来检查这些蛋白质在特定组织中的作用。我们观察到,ISX 缺乏增加了预形成和前维生素 A 的利用。我们发现,ISX 缺陷小鼠外周组织中类视黄醇的储存增加依赖于 STRA6 并受类视黄醇信号诱导。此外,双突变小鼠表现出 Stra6 突变体眼部表型的部分挽救。这种挽救是以其他组织中维生素 A 的大量积累为代价的,这表明当存在过量的维生素 A 时,它是随机分布的。值得注意的是,突变小鼠的前维生素 A 补充诱导了肝脏中 RBP 受体 2 的表达,并伴有肝酯化维生素 A 储存增加。总之,这些发现表明这种必需营养素的输送途径之间存在动态相互作用,并表明当血液中循环过量时,肝会重新摄取维生素 A。