Abstract
A biochemical analysis of glycosaminoglycans was performed in arteries of a fifteen-year old white male who died of beta thalassemia major. The patient presented the severe clinical complications resulting from hemochromatosis, which was evidenced at autopsy and by histologic examination. The arteries under study comprised the thoracic and abdominal aortas and the iliac and pulmonary arteries, which were compared with the same arteries from normal individuals. Data on total glycosaminoglycan and total collagen, including the determination of the relative contents of the different glycosaminoglycans, suggest an as yet undescribed fibrotic process in the thalassemic arteries. Also altered were the proportions of the disaccharides making up chondroitin sulfate and heparin sulfate. A reduction in the molecular weight of arterial heparin sulfate, presumably with free radical involvement, was also detected. All these changes in the extracellular matrix may be ascribed to the presence of large amounts of iron in the tissue, and as such they should be expected in other disorders with chronic iron overload.
References
Dec 1, 1979·Annals of Internal Medicine
Jul 12, 1979·Biochemical and Biophysical Research Communications·O M Toledo, P A Mourão
Sep 23, 1976·The New England Journal of Medicine·S H Orkin, D G Nathan
Feb 1, 1992·Archives of Biochemistry and Biophysics·B B Aggarwal, E Pocsik
Jan 1, 1992·Journal of Clinical Pathology·R MeliconiA Facchini
Oct 1, 1991·The British Journal of Dermatology·C W TrenamD R Blake
Jul 1, 1989·Lancet·M G ZurloS Terzoli
Jan 17, 1985·The New England Journal of Medicine·J M McCord
Mar 1, 1986·Inflammation·R A Greenwald, S A Moak
Jul 1, 1987·Chemistry and Physics of Lipids·M TsuchidaK Aibara
Oct 1, 1969·Analytical Biochemistry·J C Hilborn, P A Anastassiadis
Jan 1, 1973·Thorax·D HeathP Smith
Apr 6, 1974·British Medical Journal·M BarryR A Risdon
Aug 14, 1981·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·H A Hill
Jan 1, 1983·Critical Reviews in Clinical Laboratory Sciences·G D McLarenR W Kellermeyer
Mar 1, 1982·Archives of Biochemistry and Biophysics·T Kajiwara, M L Tanzer
Sep 10, 1994·Lancet·B Halliwell
Jun 15, 1994·The Biochemical Journal·A J DabbaghB Frei
Apr 1, 1993·Chest·P WackerM Wyss
Dec 1, 1993·Australian and New Zealand Journal of Medicine·M E RichardsonR W Harper
Mar 4, 1994·Carbohydrate Research·N Volpi
Jan 1, 1994·Annual Review of Medicine·S L Schrier
Jan 1, 1993·Research in Experimental Medicine. Zeitschrift Für Die Gesamte Experimentelle Medizin Einschliesslich Experimenteller Chirurgie·M S FigueiredoM A Zago
Oct 7, 1964·Annals of the New York Academy of Sciences·M A ENGLE
Citations
Aug 29, 2000·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·M FaillaG Mancia
Oct 7, 2006·Journal of Pediatric Hematology/oncology·Zulal UlgerArif R Ozyurek
May 17, 2005·Heart·Y F CheungG C F Chan
Nov 13, 2002·Circulation·Y F CheungS Y Ha