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| copper chaperone for SOD1, CCS ( |
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Dorfin physically bound and ubiquitylated various SOD1 mutants ( |
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NEDL1 binds and ubiquitinates mutant SOD1 ( |
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Hsp/Hsc70 interacts mutant SOD1 ( |
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anti-apoptotic protein Bcl-2 ( |
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HoxB2 binds mutant SOD1 ( |
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chromogranin-A (CgA) and chromogranin-B (CgB) interact with mutant forms of SOD1 ( |
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calcineurin, Cn ( |
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Lysyl-tRNA synthetase, KARS ( |
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gp78 ( |
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glutathione peroxidase 2, GPX2 ( |
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N-terminal portion of BCL2 between the BH4 and the loop domain |
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protects cortical neurons from hypoxia/reperfusion (I/R) injury by the increased SOD1 activity and the protective effect of MTRNR2L1 on neurons against I/R is concentration-dependent |
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CCS is the physiological partner for the complex mechanism of SOD1 maturation |
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mutant SOD1 interacting with HDAC6(likely mechanism of HDAC6 inhibition by mutant SOD1 is sequestration of HDAC6 into inclusions) |
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comprises a masked DERL1 binding region, that, under zinc-deficient conditions, adopts a mutant-like conformation exposing the DBR and inducing the homeostatic ER stress response |
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SOD1-DERL1 interaction contributes to the pathogenesis of ALS and is a promising drug target for ALS treatment |
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SOD1-interacting proteins were involved in multiple cellular functions such as protein quality control, cytoskeletal organization, and pathways involved in growth factor signaling and their downstream cascades |
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copper chaperone for SOD1 (CCS) is a dedicated and specific chaperone that assists SOD1 folding and maturation to produce the active enzyme |
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| mutations in the Sod1 Drosophila gene resulte in striking neuropathology ( | |
chronically inhibition of SOD1 by either antisense oligodeoxynucleotides or diethyldithiocarbamate in spinal cord organotypic cultures result in the apoptotic degeneration of spinal neurons, including motor neurons, over several weeks ( |
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overexpression of Sod1 in transgenic mice affects motor neurons ( |
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mice whith a incorporated transgene encoding for the human SOD1 mutation develop a form of motor neurone disease that closely resembles human forms of this disease ( |
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In the FVB/N background, mice expressing mG86R SOD1 develop an ALS phenotype at approximately 100 days. However, when these mice were bred into a mixed background of C57Bl6/129Sv, the onset of the ALS phenotype was delayed 143 days to >2 years ( |
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deregulation of Cdk5 activity associated with the hyperphosphorylation of tau and neurofilament proteins in mice expressing a mutant superoxide dismutase (G37R)) linked to amyotrophic lateral sclerosis ( |
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transgenic C. elegans strains expressing mutant human SOD1 showed vulnerability to oxidative stress ( |
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expression profiling of gene expression in SOD1-G93A transgenic mouse spinal cords indicates extensive glial activation coincident with the onset of paralysis at 3 months of age ( |
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transgenic rat model harboring the G93A mutation overexpressing the SOD1 gene result in ALS-like motor neuron disease ( |
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in G93A mice, mitochondrial respiration, electron transfer chain, and ATP synthesis were severely defective and evidence of oxidative damage to mitochondrial proteins and lipids at the time of onset of the disease ( |
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SOD1 RNA interference (RNAi) induces senescence in normal human fibroblasts dependong on p53 induction ( |
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motor neurons accumulate detergent-insoluble forms of SOD1 in transgenic mice expressing a SOD1 variant that mutates the four histidine residues that coordinately bind Cu ( |
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adipose tissue accumulation, increased energy expenditure, and concomitant skeletal hypermetabolism ALS G93A mouse ( |
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Overexpression of human SOD1 in mouse NIH3T3 fibroblasts increased SOD activity, enhanced intracellular generation of H2O2 and significantly stimulated angiogenesis by VEGF production ( |
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transgenic mice expressing SOD1-L126Z display motor neuron disease characterized by accumulation of non-ubiquitinated detergent-insoluble SOD1-L126Z in spinal cords and aggregates of SOD1-L126Z in motor neuron somatodendritic compartments ( |
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dysregulated redox stress in ALS mice caused by NADPH oxidases Nox1 and Nox2 significantly influenced the progression of motor neuron disease caused by mutant SOD1(G93A) expression ( |
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in the spinal cord of transgenic mice expressing the familial ALS SOD1 gene mutation G93A, a decrease in constitutive proteasome subunits during disease progression and an increased immunoproteasome expression were found ( |
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impairment of axonal transport of choline acetyltransferase and acetylcholine release in SOD1(G93A)-transgenic mouse ( |
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E40K missense mutation in canin SOD1 gene lead to degenerative myelopathy ( |
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G85R/WTSOD1 double transgenic mice had an acceleration of disease onset and shortened survival compared with G85R single transgenic mice, and there was an earlier appearance of pathological and immunohistochemical abnormalities ( |
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Mitochondrial respiration, electron transfer chain, and ATP synthesis were severely defective in G93A mutated hSOD1 mice and oxidative damage to mitochondrial proteins and lipids were observed |
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Misfolded SOD1 protein was found primarily within degenerating motor neurons in ALS mouse models with the human G37R, G85R, and G93A SOD1 mutations |
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Mutant SOD1 preferentially associates with the cytoplasmic face of mitochondria from spinal cords of rats and mice expressing SOD1 mutations ( |
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SOD1 total knockout (SOD1-deficient) mice display a high level of superoxide anion in the retinal ganglion cells leading to their degeneration ( |
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Mutant SOD1 mice lacking motor neuron TrkB live a month longer than controls and retain motor function for a longer period, particularly in the early phase of the disease subserved by slowed motor neuron loss, persistence of neuromuscular junction integrity and reduced astrocytic and microglial reactivity within the spinal cord |
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inhibition of Shh signaling pathway exacerbated rat ischemic damage caused by permanent middle cerebral artery occlusion, which may be correlated with down-regulated expression of Gli1, Ptch1 and Sod1 |
