Mar 6, 2016 · SOD1-G93A (congenic): This congenic line is derived from the original hybrid line by backcrossing to C57BL/6J mice. It is available through The Jackson Laboratory, Stock #004435 . Mice on a C57BL/6J background have a milder phenotype (i.e., later onset, longer lifespan) than hybrid B6SJL mice or those on an SJL background ( Pfohl et al., 2015 ...

Mar 6, 2018 · SOD1 (G37R) (congenic) - This congenic line is derived from line 29 and backcrossed to C57BL/6J. It is available through The Jackson Lab, Stock# 008229 . Some reports indicate that mice on a C57BL/6-congenic background survive about one year (e.g., Ezzi et al. 2010 ); however, according to The Jackson Lab website, mice in their colony survive ...

SOD1, which is mutated in one-fifth of people with familial amyotrophic lateral sclerosis, forms aggregates in those people and in animal models of the disease. In a paper published online this week in PNAS, scientists report on the structure of both wild-type and mutant Apo SOD1, and find that the metal-free state is disordered in solution.

Jun 4, 2019 · SOD1-G85R (congenic) - A congenic line (B6.Cg-Tg(SOD1*G85R)148Dwc/J) is available through The Jackson Lab, Stock# 008248; Cryopreserved. According to the JAX website, congenic mice have a longer lifespan (median 361 days). After the onset of motor impairment, the disease progresses rapidly, with death at 12 to 13 months of age.

Jan 16, 2016 · SOD1 aggregates in mice injected with tissue homogenates containing the SOD1-G93A “strain” (right), but not buffer (left) or homogenates from SOD1-G85R mice (center). [Courtesy of Acta Neuropathologica/Springer (Jan 2016, Vol 131, Issue 1, pp 103-114).] SOD1 Gets a Move On

Aug 8, 2018 · Moreover, aggregated SOD1 promotes more ER stress, leading to a vicious cycle that could activate harmful responses in astrocytes, he proposed. This suggests that SOD1 aggregation could play a role in the progression of sporadic disease, he said. Neil Cashman of the University of British Columbia in Vancouver agreed that this was a possibility.

new SOD1 antibodies. While this is an obvious study to do, it was not possible in the past because antibodies that are highly sensitive for misfolded SOD1 have only recently become available, Grimm said. This finding makes SOD1 a target for the entire population of some 75,000 patients in Europe, the U.S., and Japan combined, Grimm claimed.

The company then screened 2,000 SOD1 ASOs and identified two targeting the mRNA 3' untranslated region that were most effective at lowering SOD1 mRNA and protein levels in cells. Injection of ASO1 into the brains or spinal cords of mice or rats expressing mutant SOD1 reduced spinal-cord levels of SOD1 mRNA and protein.

May 29, 2009 · SOD1 is a highly conserved, ancient protein, and may be a sort of “molecular dinosaur,” Oliveberg wrote. It may now be so specialized that any mutation throws the protein off balance. “Perhaps it has painted itself into an evolutionary cul-de-sac: the function is essential, but puts a lot of ‘strain’ on the structure, so mutations are ...

Dec 17, 2014 · Scientists have characterized mice with an ALS-linked mutation in their endogenous SOD1 gene—a substitution of glycine for the aspartic acid at position 83. According to a paper in the December 2 Human Molecular Genetics, mice homozygous for the mutation developed a disease akin to early stage ALS, with dying motor neurons and degenerating ...