title: Abundance and Significance of Iron, Zinc, Copper, and Calcium in the Hearts of Patients With Friedreich Ataxia.
abstract: Cardiomyopathy is a frequent cause of death in patients with Friedreich ataxia (FA), and a characteristic pathological feature is the focal accumulation of iron (Fe) in cardiomyocytes. This restricted localization of the metal contrasts with the diffuse cardiac Fe overload in hemochromatosis and transfusion siderosis. Nevertheless, heart Fe in FA contributes to cardiomyocyte necrosis, inflammation, and scarring as the disease progresses. A putative mechanism of cardiomyopathy in FA is Fe-mediated oxidative damage. Two other transition metals zinc (Zn) and copper (Cu), are diffusely distributed throughout normal hearts and the hearts of patients with FA. The myocardium in FA is also prone to deposits of calcium in the form of scattered concretions. In this study, heart tissues (left and right ventricular walls and ventricular septum) of 23 patients with genetically confirmed FA and 8 normal controls were obtained at autopsy and analyzed for Fe, Zn, Cu, and calcium. The principal assay methods were inductively coupled plasma optical emission spectrometry and plasma mass spectrometry. Total levels of Fe in bulk extracts were not significantly higher than normal, and the concentrations of Zn also remained in the normal range. Cu levels, however, were significantly lower in FA. In conclusion, the decrease of Cu may be important in consideration of the potential benefit of Cu supplements in FA cardiomyopathy.
authors.affiliation: Laboratory of Inorganic and Nuclear Chemistry, Wadsworth Center, New York State Department of Health, Albany, New York.
authors.forename: Pamela C
authors.initials: PC
authors.lastname: Kruger
authors.affiliation: Laboratory of Inorganic and Nuclear Chemistry, Wadsworth Center, New York State Department of Health, Albany, New York; Department of Environmental Health Sciences, School of Public Health, University at Albany, State University of New York, Albany, New York.
authors.forename: Karl X
authors.initials: KX
authors.lastname: Yang
authors.affiliation: Laboratory of Inorganic and Nuclear Chemistry, Wadsworth Center, New York State Department of Health, Albany, New York; Department of Environmental Health Sciences, School of Public Health, University at Albany, State University of New York, Albany, New York.
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Parsons
authors.affiliation: Research Service, Veterans Affairs Medical Center, Albany, New York.
authors.forename: Alyssa B
authors.initials: AB
authors.lastname: Becker
authors.affiliation: Center for Neuropharmacology and Neuroscience, Albany Medical College, Albany, New York.
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Feustel
authors.affiliation: Neurology and Pathology Services, Veterans Affairs Medical Center, Albany, New York; Department of Neurology, Albany Medical College, Albany, New York; Department of Pathology, Albany Medical College, Albany, New York. Electronic address: arnulf.koeppen@med.va.gov.
authors.forename: Arnulf H
authors.initials: AH
authors.lastname: Koeppen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002118:Calcium / Q000378:metabolism*; D016022:Case-Control Studies; D002648:Child; D003300:Copper / Q000378:metabolism*; D005260:Female; D005621:Friedreich Ataxia / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism* / Q000473:pathology; D055815:Young Adult; D015032:Zinc / Q000378:metabolism*
chemical_list: D003300:Copper; D007501:Iron; D015032:Zinc; D002118:Calcium