TITLE: Commentary:
Is Alzheimer's disease iron overload of the brain?

(the full  text paper is unavailable)

JOURNAL: Alzheimer's Research

VOLUME: 03

ISSUE: 01

PAGES: 69-72

AUTHOR: Paul S. Hodgkins, John A Blair

ADDRESS: Present address: Maryland Psychiatric Research Center, University
of  Maryland School of Medicine, PO Box 21247, , Baltimore, 21228, USA.
Pharmaceutical and Biological Sciences Department, Aston University, Aston
Triangle,, Birmingham, B4 7ET, UK

ABSTRACT: Correspondence to:John Blair,Pharmaceutical and Biological
Sciences  Department, Aston University, Aston Triangle,Birmingham,B4
7ET,UKIron is an essential element for humans, however dysregulation of iron
metabolism can lead to several clinically recognised diseases, including
severe neurological disorders. Peripheral measurements of iron, ferritin,
and transferrin saturation in Alzheimer's disease and Down syndrome patients
clearly match the biochemical profile of patients with the iron overload
disorder haemochromatosis, and identifies a dysfunction in iron metabolism.
Brain iron concentrations are raised in Alzheimer's disease and its
distribution parallels affected brain areas. The chronic build up of iron in
the brain may be causally related to the reported neurotoxicity of this
element and ultimately result in the neuropathology associated with
Alzheimer's disease. Potential therapies which  involve iron chelation may
provide an effective treatment in such cases.



KEYWORDS: Alzheimer's disease Brain Iron Model Oxidation Transferrin


   J Neurochem 2000 Jul;75(1):314-20
   
Beneficial effects of dietary restriction on cerebral cortical synaptic
terminals: preservation of glucose and glutamate transport and mitochondrial
function after exposure to amyloid beta-peptide, iron, and 3-nitropropionic
acid.

    Guo Z, Ersoz A, Butterfield DA, Mattson MP
    
   Sanders-Brown Research Center on Aging. Department of Chemistry and
   Center of Membrane Sciences, University of Kentucky, Lexington,
   Kentucky, USA.
   
   Recent studies have shown that rats and mice maintained on a dietary
   restriction (DR) regimen exhibit increased resistance of neurons to
   excitotoxic, oxidative, and metabolic insults in experimental models
   of Alzheimer's, Parkinson's, and Huntington's diseases and stroke.
   Because synaptic terminals are sites where the neurodegenerative
   process may begin in such neurodegenerative disorders, we determined
   the effects of DR on synaptic homeostasis and vulnerability to
   oxidative and metabolic insults. Basal levels of glucose uptake were
   similar in cerebral cortical synaptosomes from rats maintained on DR
   for 3 months compared with synaptosomes from rats fed ad libitum.
   Exposure of synaptosomes to oxidative insults (amyloid beta-peptide
   and Fe(2+)) and a metabolic insult (the mitochondrial toxin
   3-nitropropionic acid) resulted in decreased levels of glucose uptake.
   Impairment of glucose uptake following oxidative and metabolic insults
   was significantly attenuated in synaptosomes from rats maintained on
   DR. DR was also effective in protecting synaptosomes against oxidative
   and metabolic impairment of glutamate uptake. Loss of mitochondrial
   function caused by oxidative and metabolic insults, as indicated by
   increased levels of reactive oxygen species and decreased
   transmembrane potential, was significantly attenuated in synaptosomes
   from rats maintained on DR. Levels of the stress proteins HSP-70 and
   GRP-78 were increased in synaptosomes from DR rats, consistent with
   previous data suggesting that the neuroprotective mechanism of DR
   involves a "preconditioning" effect. Collectively, our data provide
   the first evidence that DR can alter synaptic homeostasis in a manner
   that enhances the ability of synapses to withstand adversity.
   
   PMID: 10854276, UI: 20312884
     _________________________________________________________________
   
   
   Am J Med Genet 2000 Jul 3;93(1):58-66
   
Are hereditary hemochromatosis mutations involved in Alzheimer disease?

    Moalem S, Percy ME, Andrews DF, Kruck TP, Wong S, Dalton AJ, Mehta P, Fedor
    B, Warren AC
    
   Department of Physiology, University of Toronto, Toronto, ON, Canada.
   
   Mutations in the class I-like major histocompatibility complex gene
   called HFE are associated with hereditary hemochromatosis (HHC), a
   disorder of excessive iron uptake. We screened DNA samples from
   patients with familial Alzheimer disease (FAD) (n = 26), adults with
   Down syndrome (DS) (n = 50), and older (n = 41) and younger (n = 52)
   healthy normal individuals, for two HHC point mutations-C282Y and
   H63D. Because the apolipoprotein E (ApoE) E4 allele is a risk factor
   for AD and possibly also for dementia of the AD type in DS, DNA
   samples were also ApoE genotyped. Chi-squared analyses were
   interpreted at the 0.05 level of significance without Bonferroni
   corrections. In the pooled healthy normal individuals, C282Y was
   negatively associated with ApoE E4, an effect also apparent in
   individuals with DS but not with FAD. Relative to older normals, ApoE
   E4 was overrepresented in both males and females with FAD, consistent
   with ApoE E4 being a risk factor for AD; HFE mutations were
   overrepresented in males and underrepresented in females with FAD.
   Strong gender effects on the distribution of HFE mutations were
   apparent in comparisons among ApoE E4 negative individuals in the FAD
   and healthy normal groups (P < 0.002). Our findings are consistent
   with the proposition that among ApoE E4 negative individuals HFE
   mutations are predisposing to FAD in males but are somewhat protective
   in females. Further, ApoE E4 effects in our FAD group are strongest in
   females lacking HFE mutations. Relative to younger normals there was a
   tendency for ApoE E4 and H63D to be overrepresented in males and
   underrepresented in females with DS. The possibility that HFE
   mutations are important new genetic risk factors for AD should be
   pursued further. Copyright 2000 Wiley-Liss, Inc.
   
   PMID: 10861683, UI: 20321357
     _________________________________________________________________

Subject: zinc/histamine/iron/alzheimer

   Schweiz Arch Neurol Psychiatr 1990;141(6):523-56
   
[Alzheimer's dementia and zinc].

   [Article in French]
   
    Constantinidis J
    
   Universite de Geneve, Suisse.
   
   In Alzheimer's dementia (AD) the Primum Movens is Amyloid (AM)
   production on precapillaries: Dyshoric Angiopathy, and capillaries:
   Senile Plaques (SP) producing Blood-Brain-Barrier (BBB) disturbances,
   entry in the brain of toxic metals which displace the zinc. Cerebral
   AM alone may be asymptomatic. Clinical symptoms (Amnesia, Instrumental
   Disorders) appear when AM induces Neighbouring neuritic alterations:
   Paired Hellical Filaments (PHF) and Distant neuronal body lesions:
   Neurofibrillary Tangles (NFT). The AM is coded by a locus on the
   chromosome 21 and a duplication of this locus should be the etiology
   of cerebral AM in AD. In AD cerebral zinc decreases particularly in
   the hippocampus. The zinc-enzyme Superoxyde-Dismutase (SOD) is coded
   by a locus also on the chromosome 21 near AM and the plasma level of
   SOD is high in AD. Zinc deposits observed in capillary AM-SP, result
   probably from the excess of plasmatic SOD. Other metals: Iron,
   aluminium are also observed in the AM-SP and their excess in the brain
   may be related to the decrease of zinc by metal to metal displacement.
   The decrease of functional zinc in the brain may interfere in the
   pathogenesis of PHF-NFT by metalotoxicity, neighbouring and distant to
   AM. Without AM, NFT are produced also by metalotoxicity and therefore
   brain zinc displacement. a) by lead: Encephalopathia saturnica b) by
   many metals: Guam Encephalopathy c) by aluminium d) by BBB
   disturbances leading probably to an abnormal entry of metals in the
   brain (Dementia Pugilistica, viral encephalitides). NFT may be
   produced by the deficiency of the following zinc enzymes: 1. Those of
   DNA metabolism, indicating abnor (HOME) mal DNA and therefore abnormal protein
   synthesis: PHF-NFT. 2. Those of neuronal detoxication: SOD, Carbonic
   Anhydrase, Lactate Dehydrogenase leading to neuronal toxicity
   particularly in the hippocampus normally rich in SOD. 3. Of Glutamate
   (GLU) Dehydrogenase (GDH) resulting in an excitotoxic increase of GLU.
   4. Those of the metabolism of neurotransmitters (NT): neuropeptides,
   Histamine, GABA, Acetylcholine. Therapeutic proposition: a zinc
   complex crossing the BBB should be useful a) to prevent that the AM
   produces PHF-NFT by Neighbouring and Distant metalotoxicity and DNA
   changes; b) to regularise zinc-enzymes of neuronal detoxification and
   of neurotransmitters metabolisms. Preliminary trials by zinc Aspartate
   give yet promising results.
   
   Publication Types:
     * Review
     * Review, tutorial
       
   PMID: 1705360, UI: 91149894
     _________________________________________________________________
   
   Yip R, Reeves JD, Lonnerdal B, et al., "Does Iron Supplementation
   Compromise Zinc Nutrition in Healthy Infants?", (Oct 1985), The
   American Journal of Clinical Nutrition, 42 (4), pp: 683-7.
     _________________________________________________________________

Subject: zinc/DNA/iron
     _________________________________________________________________

   J. Biol. Chem. 271: 5125-5130 (1996)[96214945]

In vivo and in vitro iron-replaced zinc finger generates free radicals and
causes DNA damage.

    D. Conte, S. Narindrasorasak & B. Sarkar

   Department of Biochemistry Research, The Hospital for Sick Children,
   Toronto, Ontario M5G 1X8, Canada.

   The estrogen receptor (ER) is a ligand-activated transcription factor
   whose DNA-binding domain (ERDBD) has eight cysteines, which coordinate
   two zinc atoms, forming two zinc finger-like structures. We
   demonstrate the capability of iron to replace zinc in zinc finger
   (hereby referred to as iron finger) both in vivo (using Escherichia
   coli BL21 (DE3)) and in vitro. Iron has the ability to substitute for
   zinc in the ERDBD as demonstrated by mobility shift and methylation
   interference assays of iron finger, which show specific recognition of
   the estrogen response element. The DNA binding constants for both in
   vivo and in vitro iron-replaced zinc fingers were similar to that of
   the native finger. Atomic absorption analysis revealed a ratio of 2:1
   iron atoms/mol of ERDBD protein, as found for zinc in the crystal
   structure of native ERDBD. More importantly, we demonstrate that iron
   finger in the presence of H2O2 and ascorbate generates highly reactive
   free radicals, causing a reproducible cleavage pattern to the
   proximate DNA, the estrogen response element. The deoxyribose method,
   used to detect free radical species generated, and the resultant
   cleaved DNA ends, caused by iron finger, suggest that the free
   radicals generated are hydroxyl radicals. Due to the close proximity
   of the zinc finger to DNA, we postulate that iron-substituted zinc
   finger may generate free radicals while bound to genetic regulatory
   response elements, leading to adverse consequences such as
   iron-induced toxicity and/or carcinogenesis.

   MeSH Terms:
     * Base Sequence
     * Cloning, Molecular
     * Comparative Study
     * DNA Damage*
     * DNA-Binding Proteins/metabolism
     * DNA-Binding Proteins/biosynthesis
     * DNA, Bacterial/metabolism
     * DNA, Bacterial/chemistry
     * Escherichia coli/metabolism
     * Escherichia coli/growth & development
     * Free Radicals/metabolism
     * Human
     * Iron/pharmacology
     * Kinetics
     * Methylation
     * Models, Molecular
     * Molecular Sequence Data
     * Nucleic Acid Conformation
     * Oligodeoxyribonucleotides
     * Protein Structure, Secondary
     * Receptors, Estrogen/metabolism
     * Receptors, Estrogen/biosynthesis
     * Recombinant Proteins/metabolism
     * Recombinant Proteins/biosynthesis
     * Support, Non-U.S. Gov't
     * Time Factors
     * Zinc/physiology
     * Zinc Fingers*



   Substances:
     * Zinc
     * Iron
     * Recombinant Proteins
     * Receptors, Estrogen
     * Oligodeoxyribonucleotides
     * Free Radicals
     * DNA, Bacterial
     * DNA-Binding Proteins


     _________________________________________________________________

Subject: zinc/iron

     _________________________________________________________________
   
Effects of Trace Elements Supplementation on Measures of Nutritional Status

   Effects of supplementation with zinc (30.4 mg/d), copper (3.4 mg/d),
   and chromium (241 ug/d) on alkaline phosphatase, serum ferritin,
   hemoglobin, and plasma and erythrocyte copper, iron, and zinc were
   examined in apparently healthy adults over the age of 50. Differences
   were found between males and females within supplement groups. Serum
   ferritin decreased significantly during and following zinc
   supplementation in males. Total hemoglobin increased significantly
   from baseline to four weeks post supplementation in chromium and
   copper supplemented females. Alkaline phosphatase decreased from
   baseline to four weeks post supplementation in all groups. The
   decrease was significant in the mineral supplement groups except for
   females receiving zinc--but not in the placebo group. Plasma zinc
   increased significantly in zinc supplemented males and during
   supplementation and in copper supplemented females from the end of
   supplementation to four weeks post supplementation. In the copper
   group, plasma copper decreased during supplementation in females but
   was not significantly different over time in males, while in the
   chromium supplemented females plasma copper increased at eight weeks
   of supplementation. These results support the importance of examining
   the interactive effects of minerals in both males and females.
   
   Sponsor: Agricultural Experiment Station
   PI: Andrea Arquitt

     _________________________________________________________________


Subject: iron/alzheimer/oxidation

   
   Free Radic Res 2000 Feb;32(2):103-14
   
Time-course of oxidation of lipids in human cerebrospinal fluid in vitro.

    Arlt S, Finckh B, Beisiegel U, Kontush A
    
   Medical Clinic, University Hospital Eppendorf, Hamburg, Germany.
   
   Oxidative mechanisms play an important role in the pathogenesis of
   Alzheimer's disease, Parkinson's disease and other neurodegenerative
   diseases. To assess whether the oxidation of brain lipoproteins plays
   a role in the development of these pathologies, we investigated
   whether the lipoproteins of human cerebrospinal fluid (CSF) are
   susceptible to oxidative modification in vitro. We studied oxidation
   time-course for up to 100 h of human CSF in the absence
   (autooxidation) or presence of exogenous oxidants. Autooxidation of
   diluted CSF was found to result in a slow accumulation of lipid
   peroxidation products. The time-course of lipid hydroperoxide
   accumulation revealed three consecutive phases, lag-phase, propagation
   phase and plateau phase. Qualitatively similar time-course has been
   typically found in human plasma and plasma lipoproteins. Autooxidation
   of CSF was accelerated by adding exogenous oxidants, delayed by adding
   antioxidants and completely inhibited by adding a chelator of
   transition metal ions. Autooxidation of CSF also resulted in the
   consumption of endogenous ascorbate, alpha-tocopherol, urate and
   linoleic and arachidonic acids. Taking into account that (i) lipid
   peroxidation products measured in our study are known to be derived
   from fatty acids, and (ii) lipophilic antioxidants and fatty acids
   present in CSF are likely to be located in CSF lipoproteins, we
   conclude that lipoproteins of human CSF are modified in vitro during
   its autooxidation. This autooxidation appears to be catalyzed by
   transition metal ions, such as Cu(II) and Fe(III), which are present
   in native CSF. These data suggest that the oxidation of CSF
   lipoproteins might occur in vivo and play a role in the pathogenesis
   of neurodegenerative diseases.
   
   PMID: 10653481, UI: 20117206
     _________________________________________________________________
   
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Subject: alzheimers/chelation 

   
   J Struct Biol 2000 Jun;130(2-3):209-16 Related Articles, Books,
   LinkOut
   
   Chelation and intercalation: complementary properties in a compound
   for the treatment of Alzheimer's disease.
   
   Cherny RA, Barnham KJ, Lynch T, Volitakis I, Li QX, McLean CA,
   Multhaup G, Beyreuther K, Tanzi RE, Masters CL, Bush AI
   
   The Department of Pathology, The University of Melbourne, Melbourne,
   Victoria, 3010, Australia.
   
   [Medline record in process]
   
   Selective application of metal chelators to homogenates of human
   Alzheimer's disease (AD) brain has led us to propose that the
   architecture of aggregated beta-amyloid peptide, whether in the form
   of plaques or soluble oligomers, is determined at least in part by
   high-affinity binding of transition metals, especially copper and
   zinc. Of the two metals, copper is implicated in reactive oxygen
   species generating reactions, while zinc appears to be associated with
   conformational and antioxidant activity. We tested the copper
   chelators trientine, penicillamine, and bathophenanthroline for their
   ability to mobilize brain Abeta as measured against our benchmark
   compound bathocuproine (BC). All of these agents were effective in
   solubilizing brain Abeta, although BC was the most consistent across
   the range of AD brain tissue samples tested. Similarly, all of the
   copper chelators depleted copper in the high-speed supernatants. BC
   alone had no significant effect upon zinc levels in the soluble
   fraction. BC extraction of brain tissue from C100 transgenic mice
   (which express human Abeta but do not develop amyloid) revealed
   SDS-resistant dimers as Abeta was mobilized from the sedimentable to
   the soluble fraction. NMR analysis showed that, in addition to its
   copper chelating properties, BC interacts with Abeta to form a complex
   independent of the presence of copper. Such hybrid copper chelating
   and "chain breaking" properties may form the basis of a rational
   design for a therapy for Alzheimer's disease. Copyright 2000 Academic
   Press.
   
    PMID: 10940226, UI: 20400070
   


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