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家族性中枢性尿崩症中的血清神经垂体素

Serum Neurophysins in familial central diabetes insipidus.

作者信息

Legros J J, Crabbe J

出版信息

J Clin Endocrinol Metab. 1978 Nov;47(5):1065-72. doi: 10.1210/jcem-47-5-1065.

Abstract

Antibovine neurophysin antibodies (anti-bNpI and/or anti-bNpII) are present in certain patients with familial central diabetes insipidus; these are exogenous origin, as they are not present in patients who have not received treatment with crude posterior pituitary extracts over the years preceding the analysis. Immunoreactive neurophysins were detectable in the blood of five patients with familial central diabetes insipidus, and in two of them, the levels increased after a short period of water restriction. There is marked polymorphism of these neurophysins from one serum to another: neurophysin I was consistently absent, while neurophysin II, accessory neurophysins, and other immunoreactive substances not present in normal sera were sometimes present in variable amounts. Immunoreactive AVP was undetectable in the urine of all patients, while immunoreactive OT was found in three of them; the latter substance could, however, be arginine vasotocin. Data are presented suggesting that the association between the biosynthesis of neurophysin I and AVP on the one hand, and neurophysin II and OT on the other hand is maintained in patients with isolated AVP deficiency on the basis of a congenital defect.

摘要

抗牛神经垂体素抗体(抗bNpI和/或抗bNpII)存在于某些家族性中枢性尿崩症患者中;这些抗体来源于外源性,因为在分析前数年未接受过粗制垂体后叶提取物治疗的患者中不存在这些抗体。在5例家族性中枢性尿崩症患者的血液中可检测到免疫反应性神经垂体素,其中2例患者在短时间限水后其水平升高。这些神经垂体素在不同血清之间存在明显的多态性:神经垂体素I始终缺失,而神经垂体素II、辅助神经垂体素以及正常血清中不存在的其他免疫反应性物质有时会以不同量存在。所有患者的尿液中均未检测到免疫反应性精氨酸加压素(AVP),而在其中3例患者的尿液中发现了免疫反应性催产素(OT);然而,后一种物质可能是精氨酸血管催产素。所呈现的数据表明,基于先天性缺陷,在孤立性AVP缺乏的患者中,神经垂体素I与AVP的生物合成以及神经垂体素II与OT的生物合成之间的关联得以维持。

相似文献

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Serum Neurophysins in familial central diabetes insipidus.家族性中枢性尿崩症中的血清神经垂体素
J Clin Endocrinol Metab. 1978 Nov;47(5):1065-72. doi: 10.1210/jcem-47-5-1065.
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Familial neurohypophyseal diabetes insipidus associated with a signal peptide mutation.
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