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首页> 外文期刊>Current opinion in lipidology >Oxysterols: functional significance in fetal development and the maintenance of normal retinal function.
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Oxysterols: functional significance in fetal development and the maintenance of normal retinal function.

机译：氧固醇：在胎儿发育和维持正常视网膜功能中的功能意义。

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PURPOSE OF REVIEW: Recent findings extend the biologic activities of oxysterols as ligands for nuclear receptors to a role in morphogenesis during fetal development and to a role in the metabolism of photooxidation products of cholesterol in the retina. RECENT FINDINGS: A 1000-fold increase of the 27-hydroxy metabolite of 7-dehydrocholesterol in the plasma of children with Smith-Lemli-Opitz syndrome imply that intermediates in cholesterol synthesis follow alternate pathways of metabolism that generate novel oxysterols. A mouse model also finds an increase in sterol intermediates as the proximate cause of dysmorphisms. A role for oxysterols in the effects of Sonic hedgehog protein focuses on their role in normal fetal development. Both CYP27A1 and CYP46A1 are expressed in primate retina indicating that local metabolism of 7-ketocholesterol to nontoxic derivatives is important for preventing retinal degeneration. SUMMARY: Recent data expand the functional roles of oxysterols to fetal development and to the detoxification of oxidation products of cholesterol. This review shifts the focus of attention from studies of their ligand-binding activity to studies of animal models that indicate a number of important biologic effects during fetal development and during the aging process.
机译：审查的目的：最近的发现扩展了羟甾醇作为核受体的配体的生物学活性，使其在胎儿发育过程中在形态发生中起作用，并在视网膜中胆固醇的光氧化产物代谢中起作用。最近的发现：患有Smith-Lemli-Opitz综合征的儿童血浆中7-脱氢胆固醇的27-羟基代谢产物增加了1000倍，这表明胆固醇合成的中间体遵循新的代谢途径，生成新的氧固醇。小鼠模型还发现固醇中间体的增加是异形现象的直接原因。氧固醇在声波刺猬蛋白中的作用集中于它们在正常胎儿发育中的作用。 CYP27A1和CYP46A1均在灵长类动物视网膜中表达，表明7-酮胆固醇局部代谢为无毒衍生物对预防视网膜变性很重要。摘要：最新数据扩展了氧固醇在胎儿发育和胆固醇氧化产物排毒中的功能作用。这篇综述将注意力从对配体结合活性的研究转向对动物模型的研究，这些动物模型表明了在胎儿发育和衰老过程中许多重要的生物学作用。

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