Duodenal iron proteins in idiopathic hemochromatosis.

Duodenal iron proteins in idiopathic hemochromatosis.
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特发性血色素沉着症中的十二指肠铁蛋白。

DOI:
10.1172/jci113868
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发表时间:
1989
期刊:
The Journal of clinical investigation
影响因子:
--
通讯作者:
Cook,JD
Cook,JD
中科院分区:
--
文献类型:
--
作者:
Whittaker,P;Skikne,BS;Covell,AM;Flowers,C;Cooke,A;Lynch,SR;Cook,JD

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本研究旨在探讨正常人和特发性血色沉着症(IH)患者十二指肠铁蛋白浓度与铁吸收的关系。17例正常人十二指肠活检,其中3例轻度缺铁,7例未经治疗的IH患者。尽管血清铁蛋白升高了20倍,但IH患者对血红素和非血红素铁的吸收都增加了。用单抗免疫测定法测定粘膜标本中的转铁蛋白、富H铁蛋白和L富铁蛋白。正常人粘膜转铁蛋白浓度与铁状态或铁吸收均无相关性,表明粘膜转铁蛋白在铁吸收中不起生理学作用。IH患者粘膜转铁蛋白显著降低,可能是由于粘膜转铁蛋白受体减少所致。在正常人中,粘膜H和L铁蛋白浓度与体内铁贮存量呈正相关,与铁吸收呈负相关。在IH中,粘膜H和L铁蛋白未能与血清铁蛋白平行升高,但与铁吸收水平相适应。IH患者粘膜H/L铁蛋白的表达与正常人无明显差异。我们的发现表明,IH患者十二指肠铁蛋白的主要异常是富含H和L的铁蛋白浓度平行下降。目前尚不清楚这是吸收异常的结果还是原因。
This study was undertaken to assess the relationship between iron absorption and the concentration of duodenal iron proteins in normal subjects and patients with idiopathic hemochromatosis (IH). Biopsies were obtained endoscopically from the duodenum in 17 normal subjects, 3 of whom were mildly iron deficient, and 7 patients with untreated IH. The absorption of both heme and nonheme iron was increased in IH despite a 20-fold elevation in serum ferritin. Immunoassays using MAb were used to measure transferrin, H-rich ferritin, and L-rich ferritin in mucosal samples. Mucosal transferrin concentrations in normal subjects did not correlate with either iron status or iron absorption, indicating that mucosal transferrin plays no physiological role in iron absorption. Mucosal transferrin was significantly lower in IH, presumably because of a decrease in mucosal transferrin receptors. Mucosal H and L ferritin concentrations were directly related to body iron stores and inversely related to iron absorption in normal subjects. In IH, mucosal H and L ferritin failed to increase in parallel with the serum ferritin, but were appropriate for the level of iron absorption. The relationship of mucosal H/L ferritin in IH did not differ from that observed in normal subjects. Our findings indicate that the major abnormality in duodenal iron proteins in IH is a parallel decrease in the concentration of H- and L-rich ferritin. It is not evident whether this is the result or the cause of the absorptive abnormality.
豚鼠肠道铁结合蛋白。
DOI: 10.1016/0006-291x(76)91079-2
发表时间: 1976
影响因子: 3.1
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DOI: --
发表时间: 1986
期刊: American journal of hematology/oncology
影响因子: --
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DOI: 10.1016/0304-4165(79)90377-5
发表时间: 1979-01-01
期刊: BIOCHIMICA ET BIOPHYSICA ACTA
影响因子: --
作者:
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DOI: 10.1182/blood.v35.1.94.94
发表时间: 1970
期刊: Blood
影响因子: 20.3
作者:
R. B. Dawson;S. Rafal;L. Weintraub
通讯作者: L. Weintraub
DOI: 10.1111/j.1365-2141.1976.tb00194.x
发表时间: 1976
影响因子: 6.5
作者:
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通讯作者: U. Mack