Iron metabolism is disturbed and anti-copper treatment improves but does not normalize iron metabolism in Wilson's disease.
Copper
Iron
Wilson disease
Journal
Biometals : an international journal on the role of metal ions in biology, biochemistry, and medicine
ISSN: 1572-8773
Titre abrégé: Biometals
Pays: Netherlands
ID NLM: 9208478
Informations de publication
Date de publication:
04 2021
04 2021
Historique:
received:
07
05
2020
accepted:
26
01
2021
pubmed:
9
2
2021
medline:
15
12
2021
entrez:
8
2
2021
Statut:
ppublish
Résumé
Wilson's disease (WD) is a rare hereditary disorder of copper metabolism. Some data suggest that iron metabolism is disturbed in WD and this may affect the course of the disease. The current study aimed to determine whether anti-copper treatment could affect iron metabolism in WD. One hundred thirty-eight WD patients and 102 controls were examined. Serum ceruloplasmin and copper were measured by colorimetric enzyme assay or atomic adsorption spectroscopy, respectively. Routine and non-routine parameters of iron metabolism were measured by standard laboratory methods or enzyme immunoassay, respectively. WD patients, both newly diagnosed and treated, had less serum copper and ceruloplasmin than controls (90.0, 63.0, 22.0 mg/dL, respectively, p < 0.001); in the treated patients blood copper and ceruloplasmin were lower than in untreated patients (p < 0.001). Untreated patients (n = 39) had a higher median blood iron (126.0 vs 103.5 ug/dL, p < 0.05), ferritin (158.9 vs 47.5 ng/mL, p < 0.001), hepcidin (32, 6 vs 12.1 ng/mL, p < 0.001) and sTfR (0.8 vs. 0.7 ug/mL, p < 0.001) and lower blood transferrin (2.4 vs. 2.7 g/L, p < 0.001), TIBC (303.0 vs 338.0 ug/dL, p < 0.001), hemoglobin (13.1 vs 13.9 g/dL, p < 0.01) and RBC (4.3 vs. 4.6, p < 0.002) than controls. Treated patients (n = 99) had a significantly lower median iron (88.0 vs. 126.0 ug/dL, p < 0.001), ferritin (77.0 vs. 158.9 ng/mL, p < 0.005) and hepcidin (16.7 vs. 32.6 ng/mL, p < 001) and higher transferrin (2.8 vs. 2.4 g/L, p < 0.005), TIBC (336.0 vs 303.0 ug/dL, p < 0.001), RBC (4.8 vs. 4.3 M/L, p < 0.001) and hemoglobin (14.4 vs. 13.1 g/dL, p < 0.001) than untreated; the median iron (p < 0.005) was lower, and ferritin (p < 0.005), RBC (p < 0.005) and hepcidin (p < 0.002) were higher in them than in the control group. Changes in copper metabolism are accompanied by changes in iron metabolism in WD. Anti-copper treatment improves but does not normalize iron metabolism.
Identifiants
pubmed: 33555495
doi: 10.1007/s10534-021-00289-x
pii: 10.1007/s10534-021-00289-x
pmc: PMC7940312
doi:
Substances chimiques
Copper
789U1901C5
Iron
E1UOL152H7
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
407-414Subventions
Organisme : Polish National Science Center
ID : No N N402 471640
Références
Cell Transplant. 2006;15(1):13-22
pubmed: 16700326
Handb Clin Neurol. 2017;142:35-41
pubmed: 28433108
Gut. 2007 Jan;56(1):115-20
pubmed: 16709660
Int J Neurosci. 2011 Jan;121(1):8-15
pubmed: 20942594
Liver Int. 2012 Jan;32(1):165-70
pubmed: 22098612
J Trace Elem Med Biol. 2020 Dec;62:126637
pubmed: 32937238
J Neurol Sci. 2013 Jun 15;329(1-2):55-8
pubmed: 23597670
Neuropathol Appl Neurobiol. 2017 Oct;43(6):514-532
pubmed: 27543917
Biochem Cell Biol. 2002;80(1):1-6
pubmed: 11908632
Liver Int. 2003 Jun;23(3):139-42
pubmed: 12955875
Dig Liver Dis. 2010 Mar;42(3):216-9
pubmed: 19640812
Liver Int. 2015 Jan;35(1):215-22
pubmed: 24517502
Blood Cells Mol Dis. 2002 Nov-Dec;29(3):384-99
pubmed: 12547229
Hepatology. 2008 Jun;47(6):2089-111
pubmed: 18506894
J Lab Clin Med. 1961 Jul;58:161-8
pubmed: 13739892
Free Radic Res. 1998 May;28(5):441-50
pubmed: 9702524
J Hepatol. 2011 Oct;55(4):913-9
pubmed: 21334398
Clin Genet. 2005 Dec;68(6):524-32
pubmed: 16283883
Am J Pathol. 2006 Feb;168(2):423-34
pubmed: 16436657
Lancet. 2007 Feb 3;369(9559):397-408
pubmed: 17276780
Am J Clin Nutr. 1998 May;67(5 Suppl):972S-977S
pubmed: 9587138
Int J Biochem Cell Biol. 1995 Feb;27(2):109-22
pubmed: 7767779
AJR Am J Roentgenol. 2009 Nov;193(5):1261-7
pubmed: 19843739
Int J Biochem Cell Biol. 1998 Oct;30(10):1063-7
pubmed: 9785470
Lancet Neurol. 2014 Oct;13(10):1045-60
pubmed: 25231526
Neurol Clin. 2020 May;38(2):417-432
pubmed: 32279718