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Title: Iron content and acid phosphatase activity in hepatic parenchymal lysosomes of patients with hemochromatosis before and after phlebotomy treatment

Abstract

Lysosomal structures in liver parenchymal cells of 3 patients with iron overload and of 3 subjects without iron-storage disorders were investigated. A combination of enzyme cytochemistry--with cerium as a captive ion to demonstrate lysosomal acid phosphatase activity--and electron probe X-ray microanalysis (EPMA) was used. We were able (1) to define and quantify lysosomal structures as lysosomes, siderosomes, or residual bodies, (2) to quantify the amount of iron and cerium simultaneously in these structures, and (3) to evaluate a possible relation between iron storage and enzyme activity. With histopathologically increased iron storage, the number of siderosomes had increased at the cost of lysosomes, with a corresponding increase in acid phosphatase activity in both organelles. In histopahtologically severe iron overload, however, acid phosphatase activity was low or not detectable and most of the iron was stored in residual bodies. After phlebotomy treatment, the number of siderosomes had decreased in favor of the lysosomes, approaching values obtained in control subjects, and acid phosphatase activity was present in all iron-containing structures. In this way a relationship between iron storage and enzyme activity was established. The iron content of the individual lysosomal structures per unit area had increased with histopathologically increased iron storage and hadmore » decreased after phlebotomy treatment. From this observation, it is concluded that the iron status of the patient is not only reflected by the amount of iron-containing hepatocytes but, as well, by the iron content lysosomal unit area.« less

Authors:
; ; ; ; ;
Publication Date:
Research Org.:
Univ. of Utrecht (Netherlands)
OSTI Identifier:
7066545
Resource Type:
Journal Article
Journal Name:
Ultrastruct. Pathol.; (United States)
Additional Journal Information:
Journal Volume: 12:2
Country of Publication:
United States
Language:
English
Subject:
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.; 59 BASIC BIOLOGICAL SCIENCES; ACID PHOSPHATASE; ENZYME ACTIVITY; HEMIC DISEASES; PATHOGENESIS; IRON; METABOLISM; ELECTRON MICROPROBE ANALYSIS; LIVER; LYSOSOMES; PATIENTS; BODY; CELL CONSTITUENTS; CHEMICAL ANALYSIS; DIGESTIVE SYSTEM; DISEASES; ELEMENTS; ENZYMES; ESTERASES; GLANDS; HYDROLASES; METALS; MICROANALYSIS; ORGANOIDS; ORGANS; PHOSPHATASES; TRANSITION ELEMENTS; 560300* - Chemicals Metabolism & Toxicology; 550901 - Pathology- Tracer Techniques

Citation Formats

Cleton, M I, de Bruijn, W C, van Blokland, W T, Marx, J J, Roelofs, J M, and Rademakers, L H. Iron content and acid phosphatase activity in hepatic parenchymal lysosomes of patients with hemochromatosis before and after phlebotomy treatment. United States: N. p., 1988. Web. doi:10.3109/01913128809058215.
Cleton, M I, de Bruijn, W C, van Blokland, W T, Marx, J J, Roelofs, J M, & Rademakers, L H. Iron content and acid phosphatase activity in hepatic parenchymal lysosomes of patients with hemochromatosis before and after phlebotomy treatment. United States. https://doi.org/10.3109/01913128809058215
Cleton, M I, de Bruijn, W C, van Blokland, W T, Marx, J J, Roelofs, J M, and Rademakers, L H. 1988. "Iron content and acid phosphatase activity in hepatic parenchymal lysosomes of patients with hemochromatosis before and after phlebotomy treatment". United States. https://doi.org/10.3109/01913128809058215.
@article{osti_7066545,
title = {Iron content and acid phosphatase activity in hepatic parenchymal lysosomes of patients with hemochromatosis before and after phlebotomy treatment},
author = {Cleton, M I and de Bruijn, W C and van Blokland, W T and Marx, J J and Roelofs, J M and Rademakers, L H},
abstractNote = {Lysosomal structures in liver parenchymal cells of 3 patients with iron overload and of 3 subjects without iron-storage disorders were investigated. A combination of enzyme cytochemistry--with cerium as a captive ion to demonstrate lysosomal acid phosphatase activity--and electron probe X-ray microanalysis (EPMA) was used. We were able (1) to define and quantify lysosomal structures as lysosomes, siderosomes, or residual bodies, (2) to quantify the amount of iron and cerium simultaneously in these structures, and (3) to evaluate a possible relation between iron storage and enzyme activity. With histopathologically increased iron storage, the number of siderosomes had increased at the cost of lysosomes, with a corresponding increase in acid phosphatase activity in both organelles. In histopahtologically severe iron overload, however, acid phosphatase activity was low or not detectable and most of the iron was stored in residual bodies. After phlebotomy treatment, the number of siderosomes had decreased in favor of the lysosomes, approaching values obtained in control subjects, and acid phosphatase activity was present in all iron-containing structures. In this way a relationship between iron storage and enzyme activity was established. The iron content of the individual lysosomal structures per unit area had increased with histopathologically increased iron storage and had decreased after phlebotomy treatment. From this observation, it is concluded that the iron status of the patient is not only reflected by the amount of iron-containing hepatocytes but, as well, by the iron content lysosomal unit area.},
doi = {10.3109/01913128809058215},
url = {https://www.osti.gov/biblio/7066545}, journal = {Ultrastruct. Pathol.; (United States)},
number = ,
volume = 12:2,
place = {United States},
year = {Tue Mar 01 00:00:00 EST 1988},
month = {Tue Mar 01 00:00:00 EST 1988}
}