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特发性血色素沉着症患者十二指肠吸收上皮细胞中缺乏铁蛋白的免疫组织化学证据。

Immunohistochemical evidence for a lack of ferritin in duodenal absorptive epithelial cells in idiopathic hemochromatosis.

作者信息

Francanzani A L, Fargion S, Romano R, Piperno A, Arosio P, Ruggeri G, Ronchi G, Fiorelli G

机构信息

Istituto di Scienze Biomediche, Ospedale San Gerardo, Monza, Italy.

出版信息

Gastroenterology. 1989 Apr;96(4):1071-8. doi: 10.1016/0016-5085(89)91625-9.

Abstract

Patients with idiopathic hemochromatosis exhibit an unexplained increase in intestinal iron absorption. The aim of this work was to study immunohistochemical H- and L-ferritin distribution in duodenal mucosal cells of patients with idiopathic hemochromatosis, and of subjects with various degrees of iron loading. Biopsy sections of gastrointestinal mucosa from 24 patients with idiopathic hemochromatosis, 10 patients with secondary iron overload, 6 normal subjects, and 13 iron-deficient subjects were analyzed with monoclonal antibodies for the presence of immunohistochemical H and L ferritin types, and with Perls' stain for hemosiderin. Ferritin content of duodenal homogenates was evaluated in 5 cases. The absorptive duodenal cells were found to contain ferritin, mostly of the L type, in apical granules; these ferritin granules were present in all normal, iron-deficient, and iron-over-loaded subjects, but were absent in 21 (87%) of the patients with established idiopathic hemochromatosis. In cells other than those of the duodenal epithelium, such as lamina propria or antral mucosa, ferritin and hemosiderin contents were related to iron loading and no difference was evident between primary and secondary iron overload. These findings indicate that (a) idiopathic hemochromatosis is associated with an altered ferritin expression in the duodenal absorptive epithelial cells, (b) this alteration cannot be detected by analysis of duodenal homogenates, (c) idiopathic hemochromatosis does not affect ferritin accumulation in the other cell types analyzed, and (d) ferritin in absorptive duodenal cells may have a regulatory role in iron absorption.

摘要

特发性血色素沉着症患者表现出无法解释的肠道铁吸收增加。本研究的目的是研究特发性血色素沉着症患者以及不同程度铁负荷受试者十二指肠黏膜细胞中免疫组化H型和L型铁蛋白的分布情况。对24例特发性血色素沉着症患者、10例继发性铁过载患者、6例正常受试者和13例缺铁受试者的胃肠道黏膜活检切片,使用单克隆抗体分析免疫组化H型和L型铁蛋白的存在情况,并用普鲁士蓝染色检测含铁血黄素。对5例患者的十二指肠匀浆铁蛋白含量进行了评估。发现十二指肠吸收细胞的顶端颗粒中含有铁蛋白,主要是L型;所有正常、缺铁和铁过载受试者中均存在这些铁蛋白颗粒,但在21例(87%)确诊的特发性血色素沉着症患者中不存在。在十二指肠上皮细胞以外的其他细胞,如固有层或胃窦黏膜中,铁蛋白和含铁血黄素的含量与铁负荷相关,原发性和继发性铁过载之间无明显差异。这些发现表明:(a)特发性血色素沉着症与十二指肠吸收上皮细胞中铁蛋白表达改变有关;(b)通过分析十二指肠匀浆无法检测到这种改变;(c)特发性血色素沉着症不影响所分析的其他细胞类型中铁蛋白的积累;(d)十二指肠吸收细胞中的铁蛋白可能在铁吸收中起调节作用。

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