DECREASED ACTIVITY OF INTESTINAL AND URINARY INTRINSIC-FACTOR IN GRASBECK-IMERSLUND DISEASE

被引：35

作者：

GUEANT, JL

SAUNIER, M

GASTIN, I

SAFI, A

LAMIREAU, T

DUCLOS, B

BIGARD, MA

GRASBECK, R

机构：

[1] UNIV NANCY 1,UNIV HOSP,DEPT GASTROENTEROL,VANDOEUVRE NANCY,FRANCE

[2] UNIV HOSP BORDEAUX,DEPT PEDIAT,BORDEAUX,FRANCE

[3] UNIV HOSP HAUTEPIERRE,DEPT GASTROENTEROL,STRASBOURG,FRANCE

[4] MINERVA FDN,HELSINKI,FINLAND

来源：

GASTROENTEROLOGY | 1995年 / 108卷 / 06期

关键词：

D O I：

10.1016/0016-5085(95)90122-1

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

Background/Aims: The pathogenesis of inherited intestinal cobalamin malabsorption (Grasbeck-Imerslund disease) remains unknown. The authors studied whether the disease corresponds to a defective expression and/or function of the intrinsic factor-cobalamin receptor in the ileum. Methods: Intrinsic factor-cobalamin receptor activity was measured using radioisotope assay and gel-filtration exclusion chromatography in ileal biopsy specimens and urine concentrates from 4 patients with Grasbeck-Imerslund disease and 5 controls. Results: Receptor activity was 164 +/- 13 fmol/ mg of protein in control biopsy specimens and <2.6 fmol/mg protein in specimens from patients. The association constant was estimated to be 3.8 +/- 0.4 (nmol/ L)(-1) in controls. A dramatic decrease in receptor activity was also observed in urine concentrate from patients with an association constant of 1.9 and 3.3 (nmol/L)(-1). Isoelectrofocusing of the cross-linked intrinsic factor-cobalamin receptor complex showed an isoelectric point at 4.8 in a patient as well as in control samples. Conclusions: It is concluded that Grasbeck-Imerslund disease is related to decreased intrinsic factor-receptor activity in intestinal mucosa; the receptor assay in urine can be helpful for diagnosis.

引用

页码：1622 / 1628

页数：7

共 19 条

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