| scholarly article | Q13442814 |
| P50 | author | Neal G. Copeland | Q66818896 |
| Nancy A. Jenkins | Q66818899 | ||
| P2093 | author name string | David R Borchelt | |
| Victoria Gonzales | |||
| David Fromholt | |||
| Jiou Wang | |||
| Michael Coonfield | |||
| Hilda Slunt | |||
| P433 | issue | 21 | |
| P921 | main subject | copper | Q753 |
| amyotrophic lateral sclerosis | Q206901 | ||
| P304 | page(s) | 2753-2764 | |
| P577 | publication date | 2003-09-09 | |
| P1433 | published in | Human Molecular Genetics | Q2720965 |
| P1476 | title | Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature | |
| P478 | volume | 12 |
| Q40577106 | 3' untranslated region in a light neurofilament (NF-L) mRNA triggers aggregation of NF-L and mutant superoxide dismutase 1 proteins in neuronal cells. |
| Q37431412 | A common property of amyotrophic lateral sclerosis-associated variants: destabilization of the copper/zinc superoxide dismutase electrostatic loop |
| Q38291545 | A limited role for disulfide cross-linking in the aggregation of mutant SOD1 linked to familial amyotrophic lateral sclerosis. |
| Q36995776 | A novel variant of human superoxide dismutase 1 harboring amyotrophic lateral sclerosis-associated and experimental mutations in metal-binding residues and free cysteines lacks toxicity in vivo |
| Q37644630 | A possible therapeutic target for Lou Gehrig's disease |
| Q33742705 | A role for copper in the toxicity of zinc-deficient superoxide dismutase to motor neurons in amyotrophic lateral sclerosis |
| Q38947838 | ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1. |
| Q36114113 | Activation of the Nrf2-ARE pathway in muscle and spinal cord during ALS-like pathology in mice expressing mutant SOD1. |
| Q36278903 | Acute intermittent hypoxia induced phrenic long-term facilitation despite increased SOD1 expression in a rat model of ALS. |
| Q34383094 | Aggregation modulating elements in mutant human superoxide dismutase 1. |
| Q33742742 | Aggregation of copper-zinc superoxide dismutase in familial and sporadic ALS |
| Q27680903 | Aggregation-triggering segments of SOD1 fibril formation support a common pathway for familial and sporadic ALS |
| Q45251257 | Amyotrophic lateral sclerosis mutations have the greatest destabilizing effect on the apo- and reduced form of SOD1, leading to unfolding and oxidative aggregation. |
| Q48217709 | Amyotrophic lateral sclerosis-related mutant superoxide dismutase 1 aggregates inhibit 14-3-3-mediated cell survival by sequestration into the JUNQ compartment |
| Q37677565 | An examination of alpha B-crystallin as a modifier of SOD1 aggregate pathology and toxicity in models of familial amyotrophic lateral sclerosis. |
| Q34341120 | An examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1 |
| Q35224579 | Analysis of mutant SOD1 electrophoretic mobility by Blue Native gel electrophoresis; evidence for soluble multimeric assemblies |
| Q37794395 | Astrogliosis in amyotrophic lateral sclerosis: role and therapeutic potential of astrocytes |
| Q50546707 | Bacterial inclusion bodies of Alzheimer's disease β-amyloid peptides can be employed to study native-like aggregation intermediate states. |
| Q28483874 | Biochemical characterization and cellular effects of CADASIL mutants of NOTCH3 |
| Q35162951 | Biochemical properties and in vivo effects of the SOD1 zinc-binding site mutant (H80G) |
| Q33979028 | Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS. |
| Q37659809 | Blood-spinal cord barrier disruption contributes to early motor-neuron degeneration in ALS-model mice |
| Q35868223 | Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span |
| Q41955617 | Calcium ions promote superoxide dismutase 1 (SOD1) aggregation into non-fibrillar amyloid: a link to toxic effects of calcium overload in amyotrophic lateral sclerosis (ALS)? |
| Q45007472 | Calcium-permeable AMPA receptors promote misfolding of mutant SOD1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model |
| Q42616369 | Canine degenerative myelopathy: biochemical characterization of superoxide dismutase 1 in the first naturally occurring non-human amyotrophic lateral sclerosis model |
| Q38766626 | Cellular Redox Systems Impact the Aggregation of Cu,Zn Superoxide Dismutase Linked to Familial Amyotrophic Lateral Sclerosis |
| Q64949212 | Changes in proteome solubility indicate widespread proteostatic disruption in mouse models of neurodegenerative disease. |
| Q57261217 | Chapter 14 Familial amyotrophic lateral sclerosis |
| Q59544323 | Chapter 4 Molecular mechanisms of motor neuron degeneration in amyotrophic lateral sclerosis |
| Q90268782 | Chemogenomics Systems Pharmacology Mapping of Potential Drug Targets for Treatment of Traumatic Brain Injury |
| Q53572441 | Clinical characteristics of familial amyotrophic lateral sclerosis with a Phe20Cys mutation in the SOD1 gene in a Korean family. |
| Q39972533 | Complete loss of post-translational modifications triggers fibrillar aggregation of SOD1 in the familial form of amyotrophic lateral sclerosis |
| Q33910597 | Complex genetics of amyotrophic lateral sclerosis |
| Q42694170 | Computational methods for identifying a layered allosteric regulatory mechanism for ALS‐causing mutations of Cu‐Zn superoxide dismutase 1 |
| Q33671609 | Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases |
| Q34509452 | Copper and zinc metallation status of copper-zinc superoxide dismutase from amyotrophic lateral sclerosis transgenic mice. |
| Q83079151 | Copper chaperone-dependent and -independent activation of three copper-zinc superoxide dismutase homologs localized in different cellular compartments in Arabidopsis |
| Q34766337 | Cupric ions induce the oxidation and trigger the aggregation of human superoxide dismutase 1 |
| Q39731255 | Cysteine 111 affects aggregation and cytotoxicity of mutant Cu,Zn-superoxide dismutase associated with familial amyotrophic lateral sclerosis |
| Q36950666 | Cytosolic proteins lose solubility as amyloid deposits in a transgenic mouse model of Alzheimer-type amyloidosis |
| Q24632133 | Decreased glutathione accelerates neurological deficit and mitochondrial pathology in familial ALS-linked hSOD1(G93A) mice model |
| Q33897544 | Destabilization of apoprotein is insufficient to explain Cu,Zn-superoxide dismutase-linked ALS pathogenesis. |
| Q27312600 | Destabilizing protein polymorphisms in the genetic background direct phenotypic expression of mutant SOD1 toxicity |
| Q36512177 | Detergent-insoluble aggregates associated with amyotrophic lateral sclerosis in transgenic mice contain primarily full-length, unmodified superoxide dismutase-1. |
| Q36934610 | Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria |
| Q55003043 | Differential induction of mutant SOD1 misfolding and aggregation by tau and α-synuclein pathology. |
| Q36539434 | Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases |
| Q37375549 | Disease-associated mutations at copper ligand histidine residues of superoxide dismutase 1 diminish the binding of copper and compromise dimer stability |
| Q37677384 | Distinctive features of the D101N and D101G variants of superoxide dismutase 1; two mutations that produce rapidly progressing motor neuron disease |
| Q24544265 | Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice |
| Q98564428 | Does wild-type Cu/Zn-superoxide dismutase have pathogenic roles in amyotrophic lateral sclerosis? |
| Q35048747 | Effect of CCS on the accumulation of FALS SOD1 mutant-containing aggregates and on mitochondrial translocation of SOD1 mutants: implication of a free radical hypothesis |
| Q103002135 | Empty mesoporous silica particles significantly delay disease progression and extend survival in a mouse model of ALS |
| Q64241123 | Experimental Mutations in Superoxide Dismutase 1 Provide Insight into Potential Mechanisms Involved in Aberrant Aggregation in Familial Amyotrophic Lateral Sclerosis |
| Q53877582 | FALS mutations in Cu, Zn superoxide dismutase destabilize the dimer and increase dimer dissociation propensity: a large-scale thermodynamic analysis. |
| Q27342067 | FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration |
| Q35012498 | Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials. |
| Q48640341 | Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2-21.3. |
| Q41851111 | Features of wild-type human SOD1 limit interactions with misfolded aggregates of mouse G86R Sod1. |
| Q26741250 | From animal models to human disease: a genetic approach for personalized medicine in ALS |
| Q46426860 | GAPDH is conformationally and functionally altered in association with oxidative stress in mouse models of amyotrophic lateral sclerosis |
| Q34497123 | Genetic rodent models of amyotrophic lateral sclerosis |
| Q36210003 | HSP27 and cell survival in neurones |
| Q40150938 | Heat-shock protein 105 interacts with and suppresses aggregation of mutant Cu/Zn superoxide dismutase: clues to a possible strategy for treating ALS. |
| Q46807674 | Human SOD1 before harboring the catalytic metal: solution structure of copper-depleted, disulfide-reduced form |
| Q36187334 | Human superoxide dismutase 1 (hSOD1) maturation through interaction with human copper chaperone for SOD1 (hCCS) |
| Q33772493 | Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis. |
| Q28592161 | Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis |
| Q41969514 | Impaired post-translational folding of familial ALS-linked Cu, Zn superoxide dismutase mutants |
| Q36722167 | Improved proteostasis in the secretory pathway rescues Alzheimer's disease in the mouse |
| Q33664711 | Inflammation in ALS and SMA: sorting out the good from the evil |
| Q36978868 | Initiation and elongation in fibrillation of ALS-linked superoxide dismutase |
| Q24630353 | Insulin-like growth factor-I for the treatment of amyotrophic lateral sclerosis |
| Q40150834 | Interaction between familial amyotrophic lateral sclerosis (ALS)-linked SOD1 mutants and the dynein complex |
| Q41786636 | Interaction of amyotrophic lateral sclerosis (ALS)-related mutant copper-zinc superoxide dismutase with the dynein-dynactin complex contributes to inclusion formation |
| Q24297625 | Intracellular seeded aggregation of mutant Cu,Zn-superoxide dismutase associated with amyotrophic lateral sclerosis |
| Q52753827 | Involvement of superoxide dismutase in oxidative stress in the oriental fruit fly, Bactrocera dorsalis: molecular cloning and expression profiles. |
| Q91806159 | L3MBTL1 regulates ALS/FTD-associated proteotoxicity and quality control |
| Q58608539 | Loss of charge mutations in solvent exposed Lys residues of superoxide dismutase 1 do not induce inclusion formation in cultured cell models |
| Q33423010 | Loss of metal ions, disulfide reduction and mutations related to familial ALS promote formation of amyloid-like aggregates from superoxide dismutase |
| Q37960903 | Magnetic resonance imaging of pathological processes in rodent models of amyotrophic lateral sclerosis |
| Q37707829 | Mapping superoxide dismutase 1 domains of non-native interaction: roles of intra- and intermolecular disulfide bonding in aggregation |
| Q58125486 | Marked intrafamilial phenotypic variation in a family with SOD1 C111Y mutation |
| Q26998585 | Mechanisms of mutant SOD1 induced mitochondrial toxicity in amyotrophic lateral sclerosis |
| Q44778683 | Mechanistic aspects of hSOD1 maturation from the solution structure of Cu(I) -loaded hCCS domain 1 and analysis of disulfide-free hSOD1 mutants |
| Q37448164 | Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis |
| Q37313745 | Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1. |
| Q35540393 | Model systems of motor neuron diseases as a platform for studying pathogenic mechanisms and searching for therapeutic agents |
| Q34503763 | Modeling ALS with iPSCs reveals that mutant SOD1 misregulates neurofilament balance in motor neurons |
| Q36087591 | Modulation of SOD1 Subcellular Localization by Transfection with Wild- or Mutant-type SOD1 in Primary Neuron and Astrocyte Cultures from ALS Mice. |
| Q33570225 | Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme |
| Q35489359 | Molecular and cellular pathways of neurodegeneration in motor neurone disease |
| Q36573336 | Molecular biology of amyotrophic lateral sclerosis: insights from genetics |
| Q35027903 | Motor neuron trophic factors: Therapeutic use in ALS? |
| Q34318026 | Mutant small heat-shock protein 27 causes axonal Charcot-Marie-Tooth disease and distal hereditary motor neuropathy |
| Q33559556 | Mutation-dependent polymorphism of Cu,Zn-superoxide dismutase aggregates in the familial form of amyotrophic lateral sclerosis |
| Q33617813 | Neither serotonin nor adenosine-dependent mechanisms preserve ventilatory capacity in ALS rats |
| Q24631645 | Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model |
| Q40296867 | Novel mutations that enhance or repress the aggregation potential of SOD1. |
| Q89706496 | Nucleation and kinetics of SOD1 aggregation in human cells for ALS1 |
| Q48302583 | Oral treatment with Cu(II)(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis. |
| Q30431829 | Palmitoylation of superoxide dismutase 1 (SOD1) is increased for familial amyotrophic lateral sclerosis-linked SOD1 mutants |
| Q38962682 | Patient-Specific Induced Pluripotent Stem Cells for SOD1-Associated Amyotrophic Lateral Sclerosis Pathogenesis Studies |
| Q35125315 | Posttranslational modifications in Cu,Zn-superoxide dismutase and mutations associated with amyotrophic lateral sclerosis. |
| Q36426422 | Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways |
| Q42970220 | Prognostic role of "prion-like propagation" in SOD1-linked familial ALS: an alternative view |
| Q36565686 | Promises and challenges in developing RNAi as a research tool and therapy for neurodegenerative diseases |
| Q33356197 | Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival |
| Q38091029 | Protein misfolding in the late-onset neurodegenerative diseases: common themes and the unique case of amyotrophic lateral sclerosis. |
| Q37775036 | Proteomic analysis of mitochondrial dysfunction in neurodegenerative diseases |
| Q27312567 | RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress response |
| Q36497696 | Redox properties of the disulfide bond of human Cu,Zn superoxide dismutase and the effects of human glutaredoxin 1 |
| Q27312393 | Regulation of protein quality control by UBE4B and LSD1 through p53-mediated transcription |
| Q46537325 | Relationship between mutant Cu/Zn superoxide dismutase 1 maturation and inclusion formation in cell models |
| Q37836981 | Review: The role of mitochondria in the pathogenesis of amyotrophic lateral sclerosis. |
| Q34464612 | Role of disulfide cross-linking of mutant SOD1 in the formation of inclusion-body-like structures |
| Q37175256 | Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS |
| Q36835575 | Role of zinc in ALS. |
| Q37894600 | SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments |
| Q26785352 | SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis |
| Q27648424 | Structural Characterization of Zinc-deficient Human Superoxide Dismutase and Implications for ALS |
| Q27653849 | Structural and Biophysical Properties of the Pathogenic SOD1 Variant H46R/H48Q † |
| Q40594112 | Structural basis of Cu, Zn-superoxide dismutase amyloid fibril formation involves interaction of multiple peptide core regions |
| Q36991027 | Structural similarity of wild-type and ALS-mutant superoxide dismutase-1 fibrils using limited proteolysis and atomic force microscopy |
| Q27664114 | Structures of mouse SOD1 and human/mouse SOD1 chimeras |
| Q35418431 | Substantially elevating the levels of αB-crystallin in spinal motor neurons of mutant SOD1 mice does not significantly delay paralysis or attenuate mutant protein aggregation |
| Q35642667 | Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states |
| Q52599963 | Superoxide dismutase-1 and other proteins in inclusions from transgenic amyotrophic lateral sclerosis model mice. |
| Q34829446 | TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling |
| Q81947132 | Targeting angiogenin in therapy of amyotropic lateral sclerosis |
| Q92711469 | The Regulation of the Small Heat Shock Protein B8 in Misfolding Protein Diseases Causing Motoneuronal and Muscle Cell Death |
| Q37375579 | The effects of glutaredoxin and copper activation pathways on the disulfide and stability of Cu,Zn superoxide dismutase |
| Q41716408 | The heat shock response in neurons and astroglia and its role in neurodegenerative diseases. |
| Q28117892 | The potential of GPNMB as novel neuroprotective factor in amyotrophic lateral sclerosis |
| Q33356208 | The threat of instability: neurodegeneration predicted by protein destabilization and aggregation propensity |
| Q61807545 | Transgenic and physiological mouse models give insights into different aspects of amyotrophic lateral sclerosis |
| Q34890228 | Transgenic animal models of neurodegeneration based on human genetic studies. |
| Q37863544 | Transition metal homeostasis: from yeast to human disease. |
| Q37295060 | Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease. |
| Q36970419 | Vulnerability of newly synthesized proteins to proteostasis stress |
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