SOD1-G93A (hybrid) (G1H) | ALZFORUM
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.
SOD1, Tau Swept Up in Prion-like Biology at ALS Meeting
Jan 16, 2016 · In prion terms, SOD1-G93A and G37R would be considered different strains, Ayers pointed out. In a study published last month, Ayers reported how SOD1 misfolding spread throughout the mouse’s nervous system following injection …
Gene Editing Delays Disease Onset in ALS Model Mice
Dec 22, 2017 · The treatment did not affect expression of mouse SOD1. The researchers also sequenced hSOD1-G93A transgenes in lumbar and thoracic spinal cord tissue (which contains motor neurons in addition to non-transduced nerve and glial cells), and found a seven- and 14-fold rise in indels—CRISPR’s smoking gun.
Background and gender effects on survival in the TgN (SOD1 …
Heiman-Patterson TD, Deitch JS, Blankenhorn EP, Erwin KL, Perreault MJ, Alexander BK, Byers N, Toman I, Alexander GM. Background and gender effects on survival in the TgN (SOD1-G93A)1Gur mouse model of ALS. J Neurol Sci. 2005 Sep 15;236 (1-2):1-7. PubMed. Recommends Please login to recommend the paper.
SOD1 (G37R) | ALZFORUM
Mar 6, 2018 · Species: Mouse Genes: SOD1 Mutations: SOD1 G37R Modification: SOD1: Transgenic Disease Relevance: Amyotrophic Lateral Sclerosis Strain Name: N/A Genetic Background: C57BL/6J x C3H/HeJ)F2 Availability: Unknown Summary This early transgenic mouse model of ALS overexpresses mutant human SOD1 (Wong et al., 1995). The transgene is regulated by the endogenous human SOD1 promoter and regulatory ...
From Protector to Instigator, Autophagy Makes About-Face in ALS
Sep 15, 2017 · In SOD1-G93A ALS mice, scientists deleted the autophagy gene Atg7 in motor neurons. These mice developed ALS at a younger age but lived longer than SOD1-G93A controls. Early on, autophagy in motor neurons delayed denervation of neuromuscular junctions, but later, it stressed interneurons and inflamed glia.
Effect of genetic background on onset and disease progression in …
Mancuso R, Oliván S, Mancera P, Pastén-Zamorano A, Manzano R, Casas C, Osta R, Navarro X. Effect of genetic background on onset and disease progression in the SOD1-G93A model of amyotrophic lateral sclerosis.
C4F6 Targets Protein That Sinks Motor Neurons - ALZFORUM
Apr 4, 2012 · Solubility experiments, involving serially extracting proteins from spinal cords of late-stage SOD1-G93A animals in increasingly harsh solvents and then looking for protein recognized by C4F6 showed that it was the soluble form of SOD1 that bound the antibody.
ALS Research Models | ALZFORUM
Dozens of rodents have been generated that model various aspects of ALS, like motor impairment or degeneration of motor neurons. No model recapitulates the human disease perfectly. By organizing information related to the phenotypic characterization of selected ALS models, this resource conveys what ...
Next-Gen Antisense and Small Protein-Protein Disruptors Benefit …
Jul 19, 2018 · Next-gen antisense oligonucleotides to SOD1 more potent than first. They knocked down SOD1 expression, prolonged survival, and reversed neuromuscular problems in rodent models. Small molecule inhibitors of SOD1’s interaction with Derlin1 also rescued symptoms.