SOD1/2
Stathmin 1/2-triggered microtubule loss mediates Golgi fragmentation in mutant SOD1 motor neurons
... human SOD1. Mutant SOD1 G93A mice (line G1del) develop limb weakness between day 165 to 240 and fatal paralysis about 40 days later ...Mutant SOD1 G85R mice (line 148) develop limb weakness between ...
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In vivo kinetic approach reveals slow SOD1 turnover in the CNS
... and total protein from liver, cortex, and spinal cord were incorporated into a compartmental model. The KADDLGKGGNEE peptide was used for modeling, as it had the most robust LC/tandem MS signal. The model consisted of a ...
10
Current developments in gene therapy for amyotrophic lateral sclerosis.
... mechanisms. SOD1 fALS mutations have been shown to inhibit the glutamate uptake function of excitatory aminoacid transporter -2 (EAAT2 or GLT1 in rodents) ...the SOD1- G93A mouse model, where ...
36
Better survival in female SOD1 mutant patients with ALS: a study of SOD1 related natural history
... Only probands of families and apparently sporadic patients were included in the genetic detection analysis. Sanger se- quencing was performed for all coding exons and flanking 50 bps of SOD1 (NM_0000454.4) and ...
10
An endogenous peptide marker differentiates SOD1 stability and facilitates pharmacodynamic monitoring in SOD1 amyotrophic lateral sclerosis
... from SOD1 mutation carriers (n = 14 ALS patients, and n = 13 presymptomatic gene mutation carriers), 29 controls (with no known neurological disease), and 30 neurological disease controls (Alzheimer’s disease and ...
12
Heat shock factor 1 over-expression protects against exposure of hydrophobic residues on mutant SOD1 and early mortality in a mouse model of amyotrophic lateral sclerosis
... was SOD1, which is responsible for 20% of familial cases, and over 150 mutations in SOD1 have been described ...[3]. SOD1 mutations can be grouped into two families based on their biophysical effects ...
17
Fragment based drug discovery for SOD1-ALS and solution X-ray scattering studies on the copper chaperone for SOD1 (hCCS)and its functional complexes with SOD1
... The p(r) function of wtSOD1 in Figure 7.16B is indicative of a protein comprised of two domains in close contact with each other and a slight elongation. Similarly the distance distribution function of the ...
174
Human SOD1 ALS Mutations in a Drosophila Knock-In Model Cause Severe Phenotypes and Reveal Dosage-Sensitive Gain- and Loss-of-Function Components
... cycles. The parental flies were collected from population- density-controlled broods (2–3 males and females in each vial) to avoid any confounding effects due to overcrowding. The parents were allowed to mate and ...
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Programmed cell death in amyotrophic lateral sclerosis
... mutant SOD1 (50) but are structurally and functionally altered in transgenic mutant SOD1 mice (51, 52), and because they play a pivotal role in PCD ...mutant SOD1 model are the Western blot and ...
10
Primary glia expressing the G93A-SOD1 mutation present a neuroinflammatory phenotype and provide a cellular system for studies of glial inflammation
... human SOD1-expressing) transgenic mouse pups demonstrated a significant elevation in either the basal or the tumor necrosis alpha (TNF α )-stimulated levels of proinflammatory eicosanoids prostaglandin E 2 ...
9
SOD1 ) rs2070424, rs4998557 polymorphisms and the susceptibility of Alzheimer’s Disease (AD) in Chinese Han
... Alzheimer’s disease (AD), also known as senile dementia, is a kind of degenerative disease of the central nervous system characterized by progressive cognitive impairment and memory impairment. Clinical manifestations is ...
6
Expression of a Cu,Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of neuroblastoma cells to infectious injury
... Neuronal injury in infection can originate from systemic inflammation, stimulation of local immunocompetent cells and direct action of bacterial toxins on neurons [1,18]. During CNS infections, Streptococcus pneumo- niae ...
12
The APCs of neuroprotection
... mutant SOD1–related ALS are unknown. People with mutant SOD1–related ALS repre- sent only about 20% of inherited ...about 2% of all patients with ALS ...mutant SOD1–related ALS develop a ...
5
Rapamycin increases survival in ALS mice lacking mature lymphocytes
... −/− SOD1 G93A mice on rapamycin- containing chow (Figure ...−/− SOD1 G93A mice fed rapamycin-containing chow have a similar amount of neurons in the spinal cord at end stage (Figure 3M), suggesting these ...
5
α-synuclein interacts with SOD1 and promotes its oligomerization
... The co-immunoprecipitation (co-IP) was performed using a adapted version of the ReCLIP method (reversible cross- link immunoprecipitation) [58, 59]. In brief, H4 cells were washed 2 times with PBS 24 h post ...
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Immunochemical characterization on pathological oligomers of mutant Cu/Zn-superoxide dismutase in amyotrophic lateral sclerosis
... human SOD1-ALS cases as well as the transgenic mice (G1H and loxG37R mice) but not to the controls with- out SOD1 ...the SOD1-ALS cases (ventral horn > dorsal horn; ...(Figs. 2 and ...
18
Disrupting SOD1 activity inhibits cell growth and enhances lipid accumulation in nasopharyngeal carcinoma
... of SOD1 affects the antioxidant defence system and nasopharyngeal carcinoma cell ...Disrupting SOD1 by LCS-1 treatment or knocking down SOD1 drastically reduced cell viability, and the overexpression ...
13
Antisense oligonucleotides extend survival and reverse decrement in muscle response in ALS models
... as SOD1-related ALS may be challenging given the relatively small number of patients affected by the disease, but much of the groundwork for such a trial has been ...of SOD1 ALS have defined survival times ...
11
SOD1, an unexpected novel target for cancer therapy.
... in SOD1 are responsible for approximately 20% of fALS ...of SOD1-ALS is not completely understood, and different mechanisms may participate in pathogenesis [3], including mitochondrial dysfunction [4, ...
7
ALS-linked misfolded SOD1 species have divergent impacts on mitochondria
... misfolded SOD1 hav- ing increased potency. The misfolded SOD1 antibody DSE2-3H1 detects misfolded SOD1 interacting with Voltage-dependent anion channel 1 (VDAC1) [20], a mitochondrial outer membrane ...
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