Reference health
Insights into the mechanisms of copper dyshomeostasis in amyotrophic lateral sclerosis
119/119 checkable references clean · checked 2026-07-23
Every reference with a DOI in the deposited reference list resolved to a known work in Crossref or DataCite at the dated check, and none carried a retraction, withdrawal, or removal notice.
2 without a DOI — not checked. A reference deposited without a DOI is never matched by title or guessed at; it stays outside the checked set, and this line discloses that.
The 119 checked references that resolve
resolves10.1016/j.nbd.2015.02.023
ZnII(atsm) is protective in amyotrophic lateral sclerosis model mice via a copper delivery mechanism
resolves10.1111/j.1471-4159.2007.04604.x
The lipophilic metal chelators DP‐109 and DP‐460 are neuroprotective in a transgenic mouse model of amyotrophic lateral sclerosis
resolves10.1016/S0014-4886(02)00014-6
The efficacy of trientine or ascorbate alone compared to that of the combined treatment with these two agents in familial amyotrophic lateral sclerosis model mice
resolves10.1002/(SICI)1097-4695(200001)42:1<49::AID-NEU5>3.0.CO;2-7
Prevention of mutant SOD1 motoneuron degeneration by copper chelatorsin vitro
resolves10.1093/abbs/gmq005
Roles of zinc and copper in modulating the oxidative refolding of bovine copper, zinc superoxide dismutase
resolves10.1016/S0304-3940(99)00227-X
Benefit of a combined treatment with trientine and ascorbate in familial amyotrophic lateral sclerosis model mice
resolves10.1016/j.bmcl.2016.02.005
Ammonium tetrathiomolybdate as a water-soluble and slow-release hydrogen sulfide donor
resolves10.1016/j.expneurol.2008.05.011
Ammonium tetrathiomolybdate delays onset, prolongs survival, and slows progression of disease in a mouse model for amyotrophic lateral sclerosis
resolves10.1152/ajpcell.00233.2006
Assembly of mitochondrial cytochrome <i>c</i>-oxidase, a complicated and highly regulated cellular process
resolves10.1038/nn823
Mutant SOD1 causes motor neuron disease independent of copper chaperone–mediated copper loading
resolves10.1073/pnas.1308531111
Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypes
resolves10.1038/ng0596-43
Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury
resolves10.1074/jbc.M114.553297
The H50Q Mutation Enhances α-Synuclein Aggregation, Secretion, and Toxicity
resolves10.1523/JNEUROSCI.22-02-00365.2002
Contrasting, Species-Dependent Modulation of Copper-Mediated Neurotoxicity by the Alzheimer's Disease Amyloid Precursor Protein
resolves10.1016/j.bbadis.2007.02.011
The cellular prion protein (PrPC): Its physiological function and role in disease
resolves10.1016/j.nbd.2012.08.015
Disruption of skeletal muscle mitochondrial network genes and miRNAs in amyotrophic lateral sclerosis
resolves10.1016/j.bbamcr.2006.03.002
Copper trafficking to the mitochondrion and assembly of copper metalloenzymes
resolves10.1074/jbc.M113.482091
Parkinson Disease Protein DJ-1 Binds Metals and Protects against Metal-induced Cytotoxicity
resolves10.1016/S0891-5849(02)01092-4
CCS knockout mice establish an alternative source of copper for SOD in ALS
resolves10.1093/hmg/8.8.1451
Variation in the Biochemical/Biophysical Properties of Mutant Superoxide Dismutase 1 Enzymes and the Rate of Disease Progression in Familial Amyotrophic Lateral Sclerosis Kindreds
resolves10.1074/jbc.M112088200
Familial Amyotrophic Lateral Sclerosis-associated Mutations Decrease the Thermal Stability of Distinctly Metallated Species of Human Copper/Zinc Superoxide Dismutase
resolves10.1146/annurev.nutr.22.012502.114457
C<scp>ERULOPLASMIN</scp> M<scp>ETABOLISM AND</scp> F<scp>UNCTION</scp>
resolves10.1002/ana.20666
DJ‐1 mutations and parkinsonism‐dementia‐amyotrophic lateral sclerosis complex
resolves10.1111/bph.12476
Mitochondrial dysfunction in amyotrophic lateral sclerosis – a valid pharmacological target?
resolves10.1042/BJ20070705
Human copper transporter 2 is localized in late endosomes and lysosomes and facilitates cellular copper uptake
resolves10.1016/0006-8993(95)00063-V
Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS)
resolves10.1046/j.1471-4159.2002.01112.x
Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosis
resolves10.1073/pnas.92.7.2539
Aceruloplasminemia: molecular characterization of this disorder of iron metabolism.
resolves10.1016/j.ejmg.2016.08.011
Recent advance in the molecular genetics of Wilson disease and hereditary hemochromatosis
resolves10.1006/nbdi.2000.0299
Human Cu/Zn Superoxide Dismutase (SOD1) Overexpression in Mice Causes Mitochondrial Vacuolization, Axonal Degeneration, and Premature Motoneuron Death and Accelerates Motoneuron Disease in Mice Expressing a Familial Amyotrophic Lateral Sclerosis Mutant SOD1
resolves10.1007/s007020070007
Unaltered cytochrome oxidase, glutamate dehydrogenase and glutaminase activities in platelets from patients with Sporadic Amyotrophic Lateral Sclerosis
resolves10.1186/s40035-016-0065-1
Dysregulation of autophagy and mitochondrial function in Parkinson’s disease
resolves10.1073/pnas.0610923104
Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology
resolves10.1016/j.neuroscience.2011.05.034
Metallothionein-III prevents neuronal death and prolongs life span in amyotrophic lateral sclerosis model mice
resolves10.1016/j.bbrc.2005.12.024
Dominant role of copper in the kinetic stability of Cu/Zn superoxide dismutase
resolves10.3389/fnagi.2014.00110
Metal-deficient aggregates and diminished copper found in cells expressing SOD1 mutations that cause ALS
resolves10.1126/sciadv.1600014
Copper-induced structural conversion templates prion protein oligomerization and neurotoxicity
resolves10.3109/21678421.2013.824000
Therapeutic effects of Cu<sup>II</sup>(atsm) in the SOD1-G37R mouse model of amyotrophic lateral sclerosis
resolves10.1111/j.1471-4159.2004.02486.x
CHIP promotes proteasomal degradation of familial ALS‐linked mutant SOD1 by ubiquitinating Hsp/Hsc70
resolves10.1007/s00415-015-7737-0
Mitochondrial dysfunction in blood cells from amyotrophic lateral sclerosis patients
resolves10.1523/JNEUROSCI.3829-04.2005
Cytochrome<i>c</i>Association with the Inner Mitochondrial Membrane Is Impaired in the CNS of G93A-SOD1 Mice
resolves10.1016/j.jns.2008.09.030
Amyotrophic lateral sclerosis linked to a novel SOD1 mutation with muscle mitochondrial dysfunction
resolves10.1073/pnas.0700477104
Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models
resolves10.1042/bj20031174
Metallothionein is crucial for safe intracellular copper storage and cell survival at normal and supra-physiological exposure levels
resolves10.1074/jbc.M110.186999
Copper and Zinc Metallation Status of Copper-Zinc Superoxide Dismutase from Amyotrophic Lateral Sclerosis Transgenic Mice
resolves10.1016/S0034-5288(18)34752-0
Cytochrome Oxidase Deficiency in the Motor Neurones of Copper-deficient Lambs: a Histochemical Study
resolves10.1523/JNEUROSCI.4196-13.2014
Oral Treatment with CuII(atsm) Increases Mutant SOD1 In Vivo but Protects Motor Neurons and Improves the Phenotype of a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis
resolves10.1016/S0022-510X(01)00627-X
Early vacuolization and mitochondrial damage in motor neurons of FALS mice are not associated with apoptosis or with changes in cytochrome oxidase histochemical reactivity
resolves10.1021/tx0000623
Reconstitution of Apo-Superoxide Dismutase by Nitric Oxide-Induced Copper Transfer from Metallothioneins
resolves10.1523/JNEUROSCI.1965-11.2011
<i>In Vivo</i>Pathogenic Role of Mutant SOD1 Localized in the Mitochondrial Intermembrane Space
resolves10.1007/s13311-015-0346-x
Regulation of Intracellular Copper by Induction of Endogenous Metallothioneins Improves the Disease Course in a Mouse Model of Amyotrophic Lateral Sclerosis
resolves10.1371/journal.pone.0059005
Cu2+ Affects Amyloid-β (1–42) Aggregation by Increasing Peptide-Peptide Binding Forces
resolves10.1016/j.nbd.2012.08.010
Cellular toxicity of mutant SOD1 protein is linked to an easily soluble, non-aggregated form in vitro
resolves10.1046/j.1471-4159.1999.0731288.x
Cysteine 144 Is a Key Residue in the Copper Reduction by the β‐Amyloid Precursor Protein
resolves10.1073/pnas.95.11.6361
Chaperone-facilitated copper binding is a property common to several classes of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants
resolves10.1111/j.1742-4658.2008.06680.x
Ligand binding promotes prion protein aggregation – role of the octapeptide repeats
resolves10.1074/jbc.M112.381178
Functional Partnership of the Copper Export Machinery and Glutathione Balance in Human Cells
resolves10.1074/jbc.M112087200
Decreased Metallation and Activity in Subsets of Mutant Superoxide Dismutases Associated with Familial Amyotrophic Lateral Sclerosis
resolves10.1074/jbc.M109.043729
Metal Deficiency Increases Aberrant Hydrophobicity of Mutant Superoxide Dismutases That Cause Amyotrophic Lateral Sclerosis
resolves10.1111/j.1471-4159.2010.06658.x
DJ‐1 forms complexes with mutant SOD1 and ameliorates its toxicity
resolves10.1016/j.nbd.2016.01.020
Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SODG93A mice co-expressing the Copper-Chaperone-for-SOD
resolves10.1111/j.1471-4159.2009.06310.x
Dysregulation of intracellular copper trafficking pathway in a mouse model of mutant copper/zinc superoxide dismutase‐linked familial amyotrophic lateral sclerosis
resolves10.1016/j.parkreldis.2016.03.001
A novel homozygous DJ1 mutation causes parkinsonism and ALS in a Turkish family
resolves10.1126/science.1077209
Mutations in the
<i>DJ-1</i>
Gene Associated with Autosomal Recessive Early-Onset Parkinsonism
resolves10.1016/j.jns.2013.11.004
Mitochondrial defects in transgenic mice expressing Cu,Zn Superoxide Dismutase mutations, the role of Copper Chaperone for SOD1
resolves10.1016/j.expneurol.2011.09.020
Absence of SOD1 leads to oxidative stress in peripheral nerve and causes a progressive distal motor axonopathy
resolves10.1039/C6MT00270F
Longitudinal assessment of metal concentrations and copper isotope ratios in the G93A SOD1 mouse model of amyotrophic lateral sclerosis
resolves10.1021/bi700620r
Metalation of the Amyotrophic Lateral Sclerosis Mutant Glycine 37 to Arginine Superoxide Dismutase (SOD1) Apoprotein Restores Its Structural and Dynamical Properties in Solution to Those of Metalated Wild-Type SOD1
resolves10.1097/NEN.0000000000000004
Altered Expression of DJ-1 and PINK1 in Sporadic ALS and in the SOD1<sup>G93A</sup>ALS Mouse Model
resolves10.1074/jbc.270.50.29991
A Physiological Role for Saccharomyces cerevisiae Copper/Zinc Superoxide Dismutase in Copper Buffering
resolves10.1007/s12031-008-9138-7
DJ-1 Changes in G93A-SOD1 Transgenic Mice: Implications for Oxidative Stress in ALS
resolves10.1074/jbc.M506521200
High Affinity Binding between Copper and Full-length Prion Protein Identified by Two Different Techniques
resolves10.1001/archneurol.2010.128
Mitochondrial Respiratory Chain Dysfunction in Muscle From Patients With Amyotrophic Lateral Sclerosis
resolves10.1002/ajmg.a.20466
Novel SCO2 mutation (G1521A) presenting as a spinal muscular atrophy type I phenotype
resolves10.1093/hmg/ddt517
Overexpression of metallothionein-I, a copper-regulating protein, attenuates intracellular copper dyshomeostasis and extends lifespan in a mouse model of amyotrophic lateral sclerosis caused by mutant superoxide dismutase-1
resolves10.1371/journal.pone.0117190
DJ-1 Knockout Augments Disease Severity and Shortens Survival in a Mouse Model of ALS
resolves10.3389/fnagi.2014.00015
Increased metal content in the TDP-43A315T transgenic mouse model of frontotemporal lobar degeneration and amyotrophic lateral sclerosis
resolves10.1084/jem.20112285
The hypoxia imaging agent CuII(atsm) is neuroprotective and improves motor and cognitive functions in multiple animal models of Parkinson’s disease
resolves10.1002/humu.22099
Molecular and biochemical characterization of a unique mutation in CCS, the human copper chaperone to superoxide dismutase
resolves10.1111/j.1460-9568.1997.tb01511.x
The Copper Chelator d‐Penicillamine Delays Onset of Disease and Extends Survival in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis
resolves10.1111/j.1471-4159.2006.03619.x
Neural mitochondrial Ca<sup>2+</sup> capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn‐superoxide dismutase mutant mice
resolves10.1523/JNEUROSCI.18-09-03241.1998
Massive Mitochondrial Degeneration in Motor Neurons Triggers the Onset of Amyotrophic Lateral Sclerosis in Mice Expressing a Mutant SOD1
resolves10.1371/journal.pone.0042277
Inhibition of TDP-43 Accumulation by Bis(thiosemicarbazonato)-Copper Complexes
resolves10.1016/j.tins.2014.05.006
Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies
resolves10.1016/j.ajhg.2010.01.027
Missense Mutations in the Copper Transporter Gene ATP7A Cause X-Linked Distal Hereditary Motor Neuropathy
resolves10.1007/BF02795615
Transfer of copper from metallothionein to nonmetallothionein proteins in cultured cells
resolves10.1126/science.1134108
Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
resolves10.1002/(SICI)1097-4547(19960801)45:3<276::AID-JNR9>3.0.CO;2-A
Decreased cytochrome c oxidase activity but unchanged superoxide dismutase and glutathione peroxidase activities in the spinal cords of patients with amyotrophic lateral sclerosis
resolves10.1073/pnas.0308298101
Mechanisms for activating Cu- and Zn-containing superoxide dismutase in the absence of the CCS Cu chaperone
resolves10.1039/C6MT00099A
Endogenous Cu in the central nervous system fails to satiate the elevated requirement for Cu in a mutant SOD1 mouse model of ALS
resolves10.1038/15513
Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene
resolves10.1002/1531-8249(199911)46:5<787::AID-ANA17>3.0.CO;2-8
Mitochondrial enzyme activity in amyotrophic lateral sclerosis: Implications for the role of mitochondria in neuronal cell death
resolves10.1016/j.nbd.2013.01.001
Dysregulation of intracellular copper homeostasis is common to transgenic mice expressing human mutant superoxide dismutase-1s regardless of their copper-binding abilities
resolves10.1006/exnr.2001.7633
Effects of an Inhibitor of Poly(ADP-Ribose) Polymerase, Desmethylselegiline, Trientine, and Lipoic Acid in Transgenic ALS Mice
resolves10.1074/jbc.M111.274407
Diacetylbis(N(4)-methylthiosemicarbazonato) Copper(II) (CuII(atsm)) Protects against Peroxynitrite-induced Nitrosative Damage and Prolongs Survival in Amyotrophic Lateral Sclerosis Mouse Model
resolves10.1002/ana.10782
DJ‐1 colocalizes with tau inclusions: A link between parkinsonism and dementia
resolves10.1002/humu.20190
Identification and analysis of 21 novel disease-causing amino acid substitutions in the conserved part of ATP7A
resolves10.1038/srep42292
CuII(atsm) improves the neurological phenotype and survival of SOD1G93A mice and selectively increases enzymatically active SOD1 in the spinal cord
resolves10.1046/j.0022-3042.2001.00731.x
Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients
resolves10.1074/jbc.M708523200
Isolated Cytochrome c Oxidase Deficiency in G93A SOD1 Mice Overexpressing CCS Protein
resolves10.1523/JNEUROSCI.19-20-08866.1999
Evidence of Presynaptic Location and Function of the Prion Protein
resolves10.1007/s00775-011-0827-2
Zn- and Cu-thioneins: a functional classification for metallothioneins?
The 2 references without a DOI — listed, not checked
no DOI — not checkedIdentification of point mutations in 41 unrelated patients affected with Menkes disease
no DOI — not checkedMutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy
What this badge says. CiteStamped means the CHECKABLE references of this
work were clean at the dated check: each resolved to a known work in a public registry, and none
carried a retraction notice at that time. It says nothing about the quality, findings,
or importance of the work itself, and nothing about references deposited without a DOI.
checked 2026-07-23 — re-checked daily as this page is visited; titles and statuses come from Crossref and DataCite and are not part of the signed record
Embed this badge
Both snippets point at the live badge image and link back to this page. The badge re-renders from the daily check, so an embed never goes stale by more than a day of visits.
<a href="https://citestamp.com/citestamped/10.1017/erm.2017.9"><img src="https://citestamp.com/citestamped/10.1017/erm.2017.9/badge.svg" alt="CiteStamped reference-health badge" width="460" height="64"></a>
[](https://citestamp.com/citestamped/10.1017/erm.2017.9)