Aggregate formation in Cu,Zn superoxide dismutase-related proteins

scientific article published on 27 January 2003

Aggregate formation in Cu,Zn superoxide dismutase-related proteins is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1074/JBC.M211698200
P698PubMed publication ID12551935

P2093author name stringJeffrey D Rothstein
Jeffrey L Elliott
Marjatta Son
Bhagya Rajendran
C Dyan Cloyd
P2860cites workAtaxin-1 with an expanded glutamine tract alters nuclear matrix-associated structuresQ24322756
Crystal structure of the copper chaperone for superoxide dismutaseQ27619198
Alpha-synuclein in Lewy bodiesQ27860680
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosisQ28131805
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutationQ28246858
Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutaseQ28343973
Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injuryQ28511628
Furin proteolytically processes the heparin-binding region of extracellular superoxide dismutaseQ28569841
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutationQ29547561
Impairment of the ubiquitin-proteasome system by protein aggregationQ29614556
Experimental models of amyotrophic lateral sclerosisQ33755043
Amyotrophy in prion diseasesQ33816986
Chromosomal localization of CCS, the copper chaperone for Cu/Zn superoxide dismutaseQ33900175
Treatment with a copper-zinc chelator markedly and rapidly inhibits beta-amyloid accumulation in Alzheimer's disease transgenic mice.Q34082498
Current status of SOD1 mutations in familial amyotrophic lateral sclerosisQ34315754
Zinc and copper in the pathogenesis of amyotrophic lateral sclerosisQ34320348
Extracellular superoxide dismutase (SOD3): tissue-specific expression, genomic characterization, and computer-assisted sequence analysis of the human EC SOD geneQ34325577
The copper chaperone CCS is abundant in neurons and astrocytes in human and rodent brainQ34487153
Normal prion protein has an activity like that of superoxide dismutase.Q34505606
Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.Q35818148
Location and properties of metal-binding sites on the human prion protein.Q37104327
Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosisQ42830531
Alzheimer's disease amyloid-beta binds copper and zinc to generate an allosterically ordered membrane-penetrating structure containing superoxide dismutase-like subunitsQ43556693
Copper converts the cellular prion protein into a protease-resistant species that is distinct from the scrapie isoformQ43559600
Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissuesQ43821139
Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding siteQ44070818
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.Q46135324
Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic miceQ48373570
Neuronal alpha-synucleinopathy with severe movement disorder in mice expressing A53T human alpha-synucleinQ48581887
Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvementQ49023143
Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.Q54066632
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondriaQ54965159
Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALSQ57042894
A gain of superoxide dismutase (SOD) activity obtained with CCS, the copper metallochaperone for SOD1Q73279835
Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutaseQ73316561
Prion protein binds copper within the physiological concentration rangeQ73692926
Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loadingQ77754821
P433issue16
P407language of work or nameEnglishQ1860
P304page(s)14331-14336
P577publication date2003-01-27
P1433published inJournal of Biological ChemistryQ867727
P1476titleAggregate formation in Cu,Zn superoxide dismutase-related proteins
P478volume278

Reverse relations

cites work (P2860)
Q405771063' untranslated region in a light neurofilament (NF-L) mRNA triggers aggregation of NF-L and mutant superoxide dismutase 1 proteins in neuronal cells.
Q28828156A faulty interaction between SOD1 and hCCS in neurodegenerative disease
Q38199669A seeded propagation of Cu, Zn-superoxide dismutase aggregates in amyotrophic lateral sclerosis.
Q37878260Brief review of the role of glycogen synthase kinase-3β in amyotrophic lateral sclerosis
Q34209733Copper modulates the degradation of copper chaperone for Cu,Zn superoxide dismutase by the 26 S proteosome.
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Q40296867Novel mutations that enhance or repress the aggregation potential of SOD1.
Q35749657Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology
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Q35550999Perturbed signal transduction in neurodegenerative disorders involving aberrant protein aggregation
Q73739590Polyglutamine-expanded ataxin-1 recruits Cu/Zn-superoxide dismutase into the nucleus of HeLa cells
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