Document Detail


Binding of copper is a mechanism of homocysteine toxicity leading to COX deficiency and apoptosis in primary neurons, PC12 and SHSY-5Y cells.
MedLine Citation:
PMID:  16876425     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Children with hereditary severe hyperhomocysteinemia present with a variety of neurological impairment, and mild hyperhomocysteinemia has been associated with neurodegeneration in the elderly. The link of hyperhomocysteinemia to neurological dysfunction is unknown. We investigated mitochondrial mechanisms of homocysteine (HCys) neurotoxicity in rat dopaminergic pheochromocytoma cells, human neuroblastoma cells and primary rat cerebellar granule neurons. HCys dose dependently impaired cytochrome c oxidase (COX) activity as well as stability and induced reactive oxygen species and apoptotic cell death. We found that HCys binds the COX cofactor Cu(2+), and Cu(2+) supplementation prior to HCys treatment preserved COX activity and prevented cell death. The Cu(2+) chelating action of HCys and impairement of COX activity represent novel mechanisms of HCys neurotoxicity, which might be preventable by supplementation of Cu(2+).
Authors:
Michael Linnebank; Holger Lutz; Eva Jarre; Stefan Vielhaber; Carmen Noelker; Eduard Struys; Cornelis Jakobs; Thomas Klockgether; Bernd O Evert; Wolfram S Kunz; Ullrich Wüllner
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Publication Detail:
Type:  Journal Article     Date:  2006-07-28
Journal Detail:
Title:  Neurobiology of disease     Volume:  23     ISSN:  0969-9961     ISO Abbreviation:  Neurobiol. Dis.     Publication Date:  2006 Sep 
Date Detail:
Created Date:  2006-08-22     Completed Date:  2006-11-01     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9500169     Medline TA:  Neurobiol Dis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  725-30     Citation Subset:  IM    
Affiliation:
Department of Neurology, University Hospital Bonn, Sigmund-Freud-Str. 25, 53125 Bonn, Germany. Michael.Linnebank@ukb.uni-bonn.de
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MeSH Terms
Descriptor/Qualifier:
Animals
Animals, Newborn
Apoptosis / drug effects,  physiology
Brain / metabolism*,  physiopathology
Cells, Cultured
Chelating Agents / metabolism,  pharmacology
Copper / metabolism*,  pharmacology
Cytochrome-c Oxidase Deficiency / metabolism*
Dose-Response Relationship, Drug
Electron Transport Complex IV / antagonists & inhibitors,  metabolism
Homocysteine / metabolism,  toxicity
Humans
Hyperhomocysteinemia / complications,  metabolism*,  physiopathology
Menkes Kinky Hair Syndrome / metabolism,  physiopathology
Neurodegenerative Diseases / genetics,  metabolism*,  physiopathology
Neurons / drug effects,  metabolism*,  pathology
Neuroprotective Agents / metabolism,  pharmacology
PC12 Cells
Rats
Chemical
Reg. No./Substance:
0/Chelating Agents; 0/Neuroprotective Agents; 454-28-4/Homocysteine; 7440-50-8/Copper; EC 1.9.3.1/Electron Transport Complex IV

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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