| Type |
Details |
Score |
| Publication |
| First Author: |
Haidet-Phillips AM |
| Year: |
2013 |
| Journal: |
Exp Neurol |
| Title: |
Altered astrocytic expression of TDP-43 does not influence motor neuron survival. |
| Volume: |
250 |
|
| Pages: |
250-9 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Yanpallewar S |
| Year: |
2021 |
| Journal: |
Exp Neurol |
| Title: |
Delayed onset of inherited ALS by deletion of the BDNF receptor TrkB.T1 is non-cell autonomous. |
| Volume: |
337 |
|
| Pages: |
113576 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Wang J |
| Year: |
2005 |
| Journal: |
Hum Mol Genet |
| Title: |
Somatodendritic accumulation of misfolded SOD1-L126Z in motor neurons mediates degeneration: alphaB-crystallin modulates aggregation. |
| Volume: |
14 |
| Issue: |
16 |
| Pages: |
2335-47 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Ouali Alami N |
| Year: |
2020 |
| Journal: |
Life Sci Alliance |
| Title: |
Multiplexed chemogenetics in astrocytes and motoneurons restore blood-spinal cord barrier in ALS. |
| Volume: |
3 |
| Issue: |
11 |
|
|
•
•
•
•
•
|
| Publication |
| First Author: |
Lemieux N |
| Year: |
1992 |
| Journal: |
Cytogenet Cell Genet |
| Title: |
New gene assignments to rabbit chromosomes; implications for chromosome evolution. |
| Volume: |
61 |
| Issue: |
2 |
| Pages: |
132-4 |
|
•
•
•
•
•
|
| Protein Domain |
| Type: |
Homologous_superfamily |
| Description: |
Superoxide dismutases (SODs) are ubiquitous metalloproteins that prevent damage by oxygen-mediated free radicals by catalysing the dismutation of superoxide into molecular oxygen and hydrogen peroxide []. Superoxide is a normal by-product of aerobic respiration and is produced by a number of reactions, including oxidative phosphorylation and photosynthesis. The dismutase enzymes have a very high catalytic efficiency due to the attraction of superoxide to the ions bound at the active site [, ].There are three forms of superoxide dismutase, depending on the metal cofactor: Cu/Zn (which binds both copper and zinc), Fe and Mn types. The Fe and Mn forms are similar in their primary, secondary and tertiary structures, but are distinct from the Cu/Zn form []. Prokaryotes and protists contain Mn, Fe or both types, while most eukaryotic organisms utilise the Cu/Zn type. The Cu/Zn form has an immunoglobulin-like β-sandwich fold.Defects in the human SOD1 gene causes familial amyotrophic lateral sclerosis (Lou Gehrig's disease). Cytoplasmic and periplasmic SODs exist as dimers, whereas chloroplastic and extracellular enzymes exist as tetramers. Structural analysis supports the notion of independent functional evolution in prokaryotes (P-class) and eukaryotes (E-class) [, , , , , , , ]. |
|
•
•
•
•
•
|
| Protein Domain |
| Type: |
Domain |
| Description: |
Superoxide dismutases (SODs) are ubiquitous metalloproteins that prevent damage by oxygen-mediated free radicals by catalysing the dismutation of superoxide into molecular oxygen and hydrogen peroxide []. Superoxide is a normal by-product of aerobic respiration and is produced by a number of reactions, including oxidative phosphorylation and photosynthesis. The dismutase enzymes have a very high catalytic efficiency due to the attraction of superoxide to the ions bound at the active site [, ].There are three forms of superoxide dismutase, depending on the metal cofactor: Cu/Zn (which binds both copper and zinc), Fe and Mn types. The Fe and Mn forms are similar in their primary, secondary and tertiary structures, but are distinct from the Cu/Zn form []. Prokaryotes and protists contain Mn, Fe or both types, while most eukaryotic organisms utilise the Cu/Zn type.Defects in the human SOD1 gene causes familial amyotrophic lateral sclerosis (Lou Gehrig's disease). Cytoplasmic and periplasmic SODs exist as dimers, whereas chloroplastic and extracellular enzymes exist as tetramers. Structural analysis supports the notion of independent functional evolution in prokaryotes (P-class) and eukaryotes (E-class) [, , , , , , , ]. |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Beltran S |
| Year: |
2019 |
| Journal: |
Mol Neurodegener |
| Title: |
Network approach identifies Pacer as an autophagy protein involved in ALS pathogenesis. |
| Volume: |
14 |
| Issue: |
1 |
| Pages: |
14 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Pandeya SR |
| Year: |
2021 |
| Journal: |
PLoS One |
| Title: |
Relationships between in vivo surface and ex vivo electrical impedance myography measurements in three different neuromuscular disorder mouse models. |
| Volume: |
16 |
| Issue: |
10 |
| Pages: |
e0259071 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Heiman-Patterson TD |
| Year: |
2005 |
| Journal: |
J Neurol Sci |
| Title: |
Background and gender effects on survival in the TgN(SOD1-G93A)1Gur mouse model of ALS. |
| Volume: |
236 |
| Issue: |
1-2 |
| Pages: |
1-7 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Pehar M |
| Year: |
2014 |
| Journal: |
PLoS One |
| Title: |
Mitochondria-targeted catalase reverts the neurotoxicity of hSOD1G⁹³A astrocytes without extending the survival of ALS-linked mutant hSOD1 mice. |
| Volume: |
9 |
| Issue: |
7 |
| Pages: |
e103438 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Pan L |
| Year: |
2012 |
| Journal: |
PLoS One |
| Title: |
Different human copper-zinc superoxide dismutase mutants, SOD1G93A and SOD1H46R, exert distinct harmful effects on gross phenotype in mice. |
| Volume: |
7 |
| Issue: |
3 |
| Pages: |
e33409 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Liu JQ |
| Year: |
2004 |
| Journal: |
J Biol Chem |
| Title: |
Reoxygenation-induced constriction in murine coronary arteries: the role of endothelial NADPH oxidase (gp91phox) and intracellular superoxide. |
| Volume: |
279 |
| Issue: |
23 |
| Pages: |
24493-7 |
|
•
•
•
•
•
|
| Strain |
| Attribute String: |
congenic, mutant strain, transgenic |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Henriques A |
| Year: |
2017 |
| Journal: |
Sci Rep |
| Title: |
Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis. |
| Volume: |
7 |
| Issue: |
1 |
| Pages: |
5235 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Teuling E |
| Year: |
2008 |
| Journal: |
Hum Mol Genet |
| Title: |
A novel mouse model with impaired dynein/dynactin function develops amyotrophic lateral sclerosis (ALS)-like features in motor neurons and improves lifespan in SOD1-ALS mice. |
| Volume: |
17 |
| Issue: |
18 |
| Pages: |
2849-62 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Poon HF |
| Year: |
2005 |
| Journal: |
Free Radic Biol Med |
| Title: |
Redox proteomics analysis of oxidatively modified proteins in G93A-SOD1 transgenic mice--a model of familial amyotrophic lateral sclerosis. |
| Volume: |
39 |
| Issue: |
4 |
| Pages: |
453-62 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
McIntosh J |
| Year: |
2023 |
| Journal: |
Front Mol Neurosci |
| Title: |
Development of abnormalities at the neuromuscular junction in the SOD1-G93A mouse model of ALS: dysfunction then disruption of postsynaptic structure precede overt motor symptoms. |
| Volume: |
16 |
|
| Pages: |
1169075 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Tesla R |
| Year: |
2012 |
| Journal: |
Proc Natl Acad Sci U S A |
| Title: |
Neuroprotective efficacy of aminopropyl carbazoles in a mouse model of amyotrophic lateral sclerosis. |
| Volume: |
109 |
| Issue: |
42 |
| Pages: |
17016-21 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Perluigi M |
| Year: |
2005 |
| Journal: |
Free Radic Biol Med |
| Title: |
Proteomic analysis of 4-hydroxy-2-nonenal-modified proteins in G93A-SOD1 transgenic mice--a model of familial amyotrophic lateral sclerosis. |
| Volume: |
38 |
| Issue: |
7 |
| Pages: |
960-8 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Li C |
| Year: |
2019 |
| Journal: |
Front Mol Neurosci |
| Title: |
Decreased Glycogenolysis by miR-338-3p Promotes Regional Glycogen Accumulation Within the Spinal Cord of Amyotrophic Lateral Sclerosis Mice. |
| Volume: |
12 |
|
| Pages: |
114 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Storkebaum E |
| Year: |
2005 |
| Journal: |
Nat Neurosci |
| Title: |
Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS. |
| Volume: |
8 |
| Issue: |
1 |
| Pages: |
85-92 |
|
•
•
•
•
•
|
| Strain |
| Attribute String: |
congenic, mutant strain, transgenic |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Arbour D |
| Year: |
2015 |
| Journal: |
J Neurosci |
| Title: |
Early and persistent abnormal decoding by glial cells at the neuromuscular junction in an ALS model. |
| Volume: |
35 |
| Issue: |
2 |
| Pages: |
688-706 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Gowing G |
| Year: |
2009 |
| Journal: |
Exp Neurol |
| Title: |
Macrophage colony stimulating factor (M-CSF) exacerbates ALS disease in a mouse model through altered responses of microglia expressing mutant superoxide dismutase. |
| Volume: |
220 |
| Issue: |
2 |
| Pages: |
267-75 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Nguyen MD |
| Year: |
2004 |
| Journal: |
J Neurosci |
| Title: |
Exacerbation of motor neuron disease by chronic stimulation of innate immunity in a mouse model of amyotrophic lateral sclerosis. |
| Volume: |
24 |
| Issue: |
6 |
| Pages: |
1340-9 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Jeong SY |
| Year: |
2009 |
| Journal: |
J Neurosci |
| Title: |
Dysregulation of iron homeostasis in the CNS contributes to disease progression in a mouse model of amyotrophic lateral sclerosis. |
| Volume: |
29 |
| Issue: |
3 |
| Pages: |
610-9 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Roy D |
| Year: |
2022 |
| Journal: |
Neurochem Res |
| Title: |
Enhanced Cortical Metabolic Activity in Females and Males of a Slow Progressing Mouse Model of Amyotrophic Lateral Sclerosis. |
| Volume: |
47 |
| Issue: |
6 |
| Pages: |
1765-1777 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Martin LJ |
| Year: |
2014 |
| Journal: |
Front Cell Neurosci |
| Title: |
GNX-4728, a novel small molecule drug inhibitor of mitochondrial permeability transition, is therapeutic in a mouse model of amyotrophic lateral sclerosis. |
| Volume: |
8 |
|
| Pages: |
433 |
|
•
•
•
•
•
|
| Strain |
| Attribute String: |
congenic, transgenic |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Molendijk J |
| Year: |
2022 |
| Journal: |
Elife |
| Title: |
Proteome-wide systems genetics identifies UFMylation as a regulator of skeletal muscle function. |
| Volume: |
11 |
|
|
|
•
•
•
•
•
|
| Allele |
| Name: |
trisomy, (16C-tel), 1 Charles J Epstein |
| Allele Type: |
Spontaneous |
|
|
•
•
•
•
•
|
| Allele |
| Name: |
single-stranded DNA binding protein 3; transgene insertion 1, Hiroshi Sasaki |
| Allele Type: |
Transgenic |
| Attribute String: |
Hypomorph, Inserted expressed sequence |
|
•
•
•
•
•
|
| Allele |
| Name: |
transgene insertion 1, Raymond Roos |
| Allele Type: |
Transgenic |
| Attribute String: |
Inducible, Inserted expressed sequence, Reporter |
|
•
•
•
•
•
|
| Strain |
| Attribute String: |
transgenic, congenic, mutant strain |
|
•
•
•
•
•
|
| Strain |
| Attribute String: |
transgenic, mutant strain, coisogenic |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Dobrowolny G |
| Year: |
2015 |
| Journal: |
Front Cell Neurosci |
| Title: |
Muscle Expression of SOD1(G93A) Modulates microRNA and mRNA Transcription Pattern Associated with the Myelination Process in the Spinal Cord of Transgenic Mice. |
| Volume: |
9 |
|
| Pages: |
463 |
|
•
•
•
•
•
|
| Strain |
| Attribute String: |
mutant stock, transgenic |
|
•
•
•
•
•
|
| Strain |
| Attribute String: |
mutant stock, transgenic |
|
•
•
•
•
•
|
| Strain |
| Attribute String: |
mutant stock, transgenic |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*G86R)M3Jwg/? |
| Background: |
involves: FVB/N |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1)51Yg/Tg(SOD1)51Yg |
| Background: |
involves: BALB/c * C57BL/6J * DBA/2 |
| Zygosity: |
hm |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1)69Yg/Tg(SOD1)69Yg |
| Background: |
involves: BALB/c * C57BL/6J * DBA/2 |
| Zygosity: |
hm |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(APP)6209Kha/? Tg(SOD1)76Dpr/? |
| Background: |
involves: C3H/HeJ * C57BL/6J * FVB/N |
| Zygosity: |
cx |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*G93A)G20Gur/? |
| Background: |
involves: C57BL/6 * SJL |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*G93A)2Gur/? |
| Background: |
involves: C57BL/6 * FVB/N * SJL |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*G37R)29Dpr/? |
| Background: |
involves: C3H/HeJ * C57BL/6 * C57BL/6J |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*G127X)716Mrkl/? |
| Background: |
B6JBom.Cg-Tg(SOD1*G127X)716Mrkl |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*G127X)832Mrkl/? |
| Background: |
involves: C57BL/6 * CBA |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*G127X)832Mrkl/Tg(SOD1*G127X)832Mrkl |
| Background: |
involves: C57BL/6 * CBA |
| Zygosity: |
hm |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*A4V)A1073Gur/? |
| Background: |
involves: C57BL/6 * SJL |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(Prnp-CCS)17Jlel/? Tg(SOD1)2Gur/? |
| Background: |
involves: C57BL/6 * SJL |
| Zygosity: |
cx |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*H46R*C111S)QAra/? |
| Background: |
involves: C57BL/6 * DBA/2 |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*H46R*C111S)PAra/? |
| Background: |
involves: C57BL/6 * DBA/2 |
| Zygosity: |
ot |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Genotype |
| Symbol: |
Tg(SOD1*H46R*C111S)PAra/Tg(SOD1*H46R*C111S)PAra |
| Background: |
involves: C57BL/6 * DBA/2 |
| Zygosity: |
hm |
| Has Mutant Allele: |
true |
|
•
•
•
•
•
|
| Protein Coding Gene |
| Type: |
protein_coding_gene |
| Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
| Protein Coding Gene |
| Type: |
protein_coding_gene |
| Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Martin LJ |
| Year: |
2019 |
| Journal: |
Front Neurol |
| Title: |
Neonatal Brain Injury and Genetic Causes of Adult-Onset Neurodegenerative Disease in Mice Interact With Effects on Acute and Late Outcomes. |
| Volume: |
10 |
|
| Pages: |
635 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Martineau É |
| Year: |
2020 |
| Journal: |
J Neurosci |
| Title: |
Properties of Glial Cell at the Neuromuscular Junction Are Incompatible with Synaptic Repair in the SOD1G37R ALS Mouse Model. |
| Volume: |
40 |
| Issue: |
40 |
| Pages: |
7759-7777 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Martin LJ |
| Year: |
2005 |
| Journal: |
J Neurosci |
| Title: |
Adult motor neuron apoptosis is mediated by nitric oxide and Fas death receptor linked by DNA damage and p53 activation. |
| Volume: |
25 |
| Issue: |
27 |
| Pages: |
6449-59 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Genç B |
| Year: |
2022 |
| Journal: |
Gene Ther |
| Title: |
Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons. |
| Volume: |
29 |
| Issue: |
3-4 |
| Pages: |
178-192 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Starikov L |
| Year: |
2020 |
| Journal: |
Neuroscience |
| Title: |
Diminished Ventral Oligodendrocyte Precursor Generation Results in the Subsequent Over-production of Dorsal Oligodendrocyte Precursors of Aberrant Morphology and Function. |
| Volume: |
450 |
|
| Pages: |
15-28 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Okuno Y |
| Year: |
2018 |
| Journal: |
Diabetes |
| Title: |
Oxidative Stress Inhibits Healthy Adipose Expansion Through Suppression of SREBF1-Mediated Lipogenic Pathway. |
| Volume: |
67 |
| Issue: |
6 |
| Pages: |
1113-1127 |
|
•
•
•
•
•
|
| Protein |
| Organism: |
Mus musculus/domesticus |
| Length: |
154
 |
| Fragment?: |
false |
|
•
•
•
•
•
|
| Protein |
| Organism: |
Mus musculus/domesticus |
| Length: |
228
 |
| Fragment?: |
false |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Banci L |
| Year: |
2002 |
| Journal: |
Eur J Biochem |
| Title: |
The solution structure of reduced dimeric copper zinc superoxide dismutase. The structural effects of dimerization. |
| Volume: |
269 |
| Issue: |
7 |
| Pages: |
1905-15 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Dzhashiashvili Y |
| Year: |
2019 |
| Journal: |
Neurobiol Dis |
| Title: |
The UPR-PERK pathway is not a promising therapeutic target for mutant SOD1-induced ALS. |
| Volume: |
127 |
|
| Pages: |
527-544 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Mouton-Liger F |
| Year: |
2011 |
| Journal: |
J Comp Neurol |
| Title: |
PCP4 (PEP19) overexpression induces premature neuronal differentiation associated with Ca(2+) /calmodulin-dependent kinase II-δ activation in mouse models of Down syndrome. |
| Volume: |
519 |
| Issue: |
14 |
| Pages: |
2779-802 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
DeRubertis FR |
| Year: |
2004 |
| Journal: |
Diabetes |
| Title: |
Attenuation of renal injury in db/db mice overexpressing superoxide dismutase: evidence for reduced superoxide-nitric oxide interaction. |
| Volume: |
53 |
| Issue: |
3 |
| Pages: |
762-8 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Sampei K |
| Year: |
2000 |
| Journal: |
Stroke |
| Title: |
Stroke outcome in double-mutant antioxidant transgenic mice. |
| Volume: |
31 |
| Issue: |
11 |
| Pages: |
2685-91 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Peviani M |
| Year: |
2007 |
| Journal: |
Mol Cell Neurosci |
| Title: |
Lack of changes in the PI3K/AKT survival pathway in the spinal cord motor neurons of a mouse model of familial amyotrophic lateral sclerosis. |
| Volume: |
34 |
| Issue: |
4 |
| Pages: |
592-602 |
|
•
•
•
•
•
|
| Publication |
| First Author: |
Vlug AS |
| Year: |
2005 |
| Journal: |
Eur J Neurosci |
| Title: |
ATF3 expression precedes death of spinal motoneurons in amyotrophic lateral sclerosis-SOD1 transgenic mice and correlates with c-Jun phosphorylation, CHOP expression, somato-dendritic ubiquitination and Golgi fragmentation. |
| Volume: |
22 |
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8 |
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