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Copper accumulation in brain

MedGen UID:
868393
•Concept ID:
C4022787
•
Finding
Synonym: Brain copper accumulation
 
HPO: HP:0012676

Definition

An anomalous build up of copper (Cu) in the brain. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCopper accumulation in brain

Professional guidelines

PubMed

Wilson's disease- management and long term outcomes.
Socha P, Czlonkowska A, Janczyk W, Litwin T
Best Pract Res Clin Gastroenterol 2022 Feb-Mar;56-57:101768. Epub 2021 Oct 12 doi: 10.1016/j.bpg.2021.101768. PMID: 35331405
Neurologic manifestations, diagnosis and management of Wilson's disease in children - an update.
Alam ST, Rahman MM, Islam KA, Ferdouse Z
Mymensingh Med J 2014 Jan;23(1):195-203. PMID: 24584398
Treatment of dystonia.
Jankovic J
Lancet Neurol 2006 Oct;5(10):864-72. doi: 10.1016/S1474-4422(06)70574-9. PMID: 16987733

Recent clinical studies

Etiology

Consequences of Disturbing Manganese Homeostasis.
Baj J, Flieger W, Barbachowska A, Kowalska B, Flieger M, Forma A, Teresiński G, Portincasa P, Buszewicz G, Radzikowska-Büchner E, Flieger J
Int J Mol Sci 2023 Oct 6;24(19) doi: 10.3390/ijms241914959. PMID: 37834407Free PMC Article
Neurological worsening in Wilson disease - clinical classification and outcome.
Mohr I, Pfeiffenberger J, Eker E, Merle U, Poujois A, Ala A, Weiss KH
J Hepatol 2023 Aug;79(2):321-328. Epub 2023 Apr 26 doi: 10.1016/j.jhep.2023.04.007. PMID: 37116715
Agricultural Use of Copper and Its Link to Alzheimer's Disease.
Coelho FC, Squitti R, Ventriglia M, Cerchiaro G, Daher JP, Rocha JG, Rongioletti MCA, Moonen AC
Biomolecules 2020 Jun 12;10(6) doi: 10.3390/biom10060897. PMID: 32545484Free PMC Article
Metal Toxicity Links to Alzheimer's Disease and Neuroinflammation.
Huat TJ, Camats-Perna J, Newcombe EA, Valmas N, Kitazawa M, Medeiros R
J Mol Biol 2019 Apr 19;431(9):1843-1868. Epub 2019 Jan 18 doi: 10.1016/j.jmb.2019.01.018. PMID: 30664867Free PMC Article
Vitamin C, Aging and Alzheimer's Disease.
Monacelli F, Acquarone E, Giannotti C, Borghi R, Nencioni A
Nutrients 2017 Jun 27;9(7) doi: 10.3390/nu9070670. PMID: 28654021Free PMC Article

Diagnosis

Wilson's disease: update on pathogenesis, biomarkers and treatments.
Shribman S, Poujois A, Bandmann O, Czlonkowska A, Warner TT
J Neurol Neurosurg Psychiatry 2021 Oct;92(10):1053-1061. Epub 2021 Aug 2 doi: 10.1136/jnnp-2021-326123. PMID: 34341141
Astrocytes in heavy metal neurotoxicity and neurodegeneration.
Li B, Xia M, Zorec R, Parpura V, Verkhratsky A
Brain Res 2021 Feb 1;1752:147234. Epub 2021 Jan 5 doi: 10.1016/j.brainres.2020.147234. PMID: 33412145Free PMC Article
Copper and the brain noradrenergic system.
Lutsenko S, Washington-Hughes C, Ralle M, Schmidt K
J Biol Inorg Chem 2019 Dec;24(8):1179-1188. Epub 2019 Nov 5 doi: 10.1007/s00775-019-01737-3. PMID: 31691104Free PMC Article
Wilson disease.
Członkowska A, Litwin T, Dusek P, Ferenci P, Lutsenko S, Medici V, Rybakowski JK, Weiss KH, Schilsky ML
Nat Rev Dis Primers 2018 Sep 6;4(1):21. doi: 10.1038/s41572-018-0018-3. PMID: 30190489Free PMC Article
Wilson's Disease.
Pfeiffer RF
Semin Neurol 2007 Apr;27(2):123-32. doi: 10.1055/s-2007-971173. PMID: 17390257

Therapy

The metal ion hypothesis of Alzheimer's disease and the anti-neuroinflammatory effect of metal chelators.
Chen LL, Fan YG, Zhao LX, Zhang Q, Wang ZY
Bioorg Chem 2023 Feb;131:106301. Epub 2022 Nov 25 doi: 10.1016/j.bioorg.2022.106301. PMID: 36455485
Astrocytes in heavy metal neurotoxicity and neurodegeneration.
Li B, Xia M, Zorec R, Parpura V, Verkhratsky A
Brain Res 2021 Feb 1;1752:147234. Epub 2021 Jan 5 doi: 10.1016/j.brainres.2020.147234. PMID: 33412145Free PMC Article
Metal Toxicity Links to Alzheimer's Disease and Neuroinflammation.
Huat TJ, Camats-Perna J, Newcombe EA, Valmas N, Kitazawa M, Medeiros R
J Mol Biol 2019 Apr 19;431(9):1843-1868. Epub 2019 Jan 18 doi: 10.1016/j.jmb.2019.01.018. PMID: 30664867Free PMC Article
Wilson disease.
Członkowska A, Litwin T, Dusek P, Ferenci P, Lutsenko S, Medici V, Rybakowski JK, Weiss KH, Schilsky ML
Nat Rev Dis Primers 2018 Sep 6;4(1):21. doi: 10.1038/s41572-018-0018-3. PMID: 30190489Free PMC Article
Vitamin C, Aging and Alzheimer's Disease.
Monacelli F, Acquarone E, Giannotti C, Borghi R, Nencioni A
Nutrients 2017 Jun 27;9(7) doi: 10.3390/nu9070670. PMID: 28654021Free PMC Article

Prognosis

Sleep Disorders in Wilson's Disease.
Cochen De Cock V, Woimant F, Poujois A
Curr Neurol Neurosci Rep 2019 Nov 13;19(11):84. doi: 10.1007/s11910-019-1001-4. PMID: 31720864
Metal Toxicity Links to Alzheimer's Disease and Neuroinflammation.
Huat TJ, Camats-Perna J, Newcombe EA, Valmas N, Kitazawa M, Medeiros R
J Mol Biol 2019 Apr 19;431(9):1843-1868. Epub 2019 Jan 18 doi: 10.1016/j.jmb.2019.01.018. PMID: 30664867Free PMC Article
Wilson disease.
Członkowska A, Litwin T, Dusek P, Ferenci P, Lutsenko S, Medici V, Rybakowski JK, Weiss KH, Schilsky ML
Nat Rev Dis Primers 2018 Sep 6;4(1):21. doi: 10.1038/s41572-018-0018-3. PMID: 30190489Free PMC Article
Hepatic features of Wilson disease.
Boga S, Ala A, Schilsky ML
Handb Clin Neurol 2017;142:91-99. doi: 10.1016/B978-0-444-63625-6.00009-4. PMID: 28433114
The neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's diseases.
Dusek P, Roos PM, Litwin T, Schneider SA, Flaten TP, Aaseth J
J Trace Elem Med Biol 2015;31:193-203. Epub 2014 Jun 2 doi: 10.1016/j.jtemb.2014.05.007. PMID: 24954801

Clinical prediction guides

Consequences of Disturbing Manganese Homeostasis.
Baj J, Flieger W, Barbachowska A, Kowalska B, Flieger M, Forma A, Teresiński G, Portincasa P, Buszewicz G, Radzikowska-Büchner E, Flieger J
Int J Mol Sci 2023 Oct 6;24(19) doi: 10.3390/ijms241914959. PMID: 37834407Free PMC Article
Neurological worsening in Wilson disease - clinical classification and outcome.
Mohr I, Pfeiffenberger J, Eker E, Merle U, Poujois A, Ala A, Weiss KH
J Hepatol 2023 Aug;79(2):321-328. Epub 2023 Apr 26 doi: 10.1016/j.jhep.2023.04.007. PMID: 37116715
Wilson's disease: update on pathogenesis, biomarkers and treatments.
Shribman S, Poujois A, Bandmann O, Czlonkowska A, Warner TT
J Neurol Neurosurg Psychiatry 2021 Oct;92(10):1053-1061. Epub 2021 Aug 2 doi: 10.1136/jnnp-2021-326123. PMID: 34341141
Metal Toxicity Links to Alzheimer's Disease and Neuroinflammation.
Huat TJ, Camats-Perna J, Newcombe EA, Valmas N, Kitazawa M, Medeiros R
J Mol Biol 2019 Apr 19;431(9):1843-1868. Epub 2019 Jan 18 doi: 10.1016/j.jmb.2019.01.018. PMID: 30664867Free PMC Article
Wilson's Disease.
Ferenci P
Clin Gastroenterol Hepatol 2005 Aug;3(8):726-33. doi: 10.1016/s1542-3565(05)00484-2. PMID: 16233999

Recent systematic reviews

Brain microstructural abnormalities in patients with Wilson's disease: A systematic review of diffusion tenor imaging studies.
Karimi A, Mohammadi S, Salehi MA, Dager SR
Brain Imaging Behav 2022 Dec;16(6):2809-2840. Epub 2022 Nov 3 doi: 10.1007/s11682-022-00733-7. PMID: 36327021
Biochemical testing for the diagnosis of Wilson's disease: A systematic review.
Salman HM, Amin M, Syed J, Sarfraz Z, Sarfraz A, Sarfraz M, Farfán Bajaña MJ, Felix M, Cherrez-Ojeda I
J Clin Lab Anal 2022 Feb;36(2):e24191. Epub 2021 Dec 23 doi: 10.1002/jcla.24191. PMID: 34951059Free PMC Article
Autonomic nervous system dysfunction in Wilson's disease - A systematic literature review.
Kłysz B, Bembenek J, Skowrońska M, Członkowska A, Kurkowska-Jastrzębska I
Auton Neurosci 2021 Dec;236:102890. Epub 2021 Oct 5 doi: 10.1016/j.autneu.2021.102890. PMID: 34656966
TMS-induced motor evoked potentials in Wilson's disease: a systematic literature review.
Bembenek JP, Kurczych K, Członkowska A
Bioelectromagnetics 2015 Apr;36(4):255-66. Epub 2015 Mar 23 doi: 10.1002/bem.21909. PMID: 25808411

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