Source Paper
Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice
Margaret Wong, Lee J. Martin
Human Molecular Genetics • 2010
Transgenic Mouse Generation and Characterization
Objective: Creation and phenotypic characterization of transgenic mice expressing wild-type, G37R, and G93A human SOD1 variants specifically in skeletal muscle to model ALS and demonstrate that skeletal muscle is a primary site of pathogenesis triggering motor neuron degeneration
This is a Transgenic Mouse Generation and Characterization protocol using mouse as the model organism. The procedure involves 4 procedural steps, 1 materials. Extracted from a 2010 paper published in Human Molecular Genetics.
Model and subjects
mouse • transgenic mice • unknown • age-related • not specified
Study window
Estimated timing pending
Core workflow
Transgenic mouse generation • Phenotypic characterization - neurologic assessment • Skeletal muscle pathology assessment
Primary readouts
- Age-related neurologic phenotypes consistent with ALS
- Limb weakness and paresis
- Motor deficits
- Skeletal muscle oxidative damage
Key equipment and reagents
Verified items
0
Direct vendor links
0
Use this page as an execution guide, then fall back to the source paper whenever you need exact exclusions, dosing details, or assay-specific caveats.
Confirm first
- Verify the animal model, intervention setup, and collection timepoints against the source paper.
- Check that every direct vendor link matches the exact specification your lab plans to run.
Use the page like this
- Work through the protocol steps in order and use the inline vendor chips only when you need to source or verify an item.
- Jump to Experimental Context for readouts, data shape, and analysis flow before planning downstream analysis.
Protocol Steps
Start here. The step list is optimized for running the experiment, with direct vendor links available inline when you need to source a cited item.
Transgenic mouse generation
Created transgenic mice expressing wild-type, G37R, and G93A human SOD1 gene variants with skeletal muscle-restricted expression
Note: Expression restricted to skeletal muscle tissue only
View evidence from paper
“We created transgenic (tg) mice expressing wild-type-, G37R- and G93A-hSOD1 gene variants only in skeletal muscle”
Phenotypic characterization - neurologic assessment
Evaluated transgenic mice for age-related neurologic phenotypes including limb weakness, paresis, and motor deficits
Note: Affected mice showed limb weakness and paresis with motor deficits
View evidence from paper
“These tg mice developed age-related neurologic and pathologic phenotypes consistent with ALS. Affected mice showed limb weakness and paresis with motor deficits”
Skeletal muscle pathology assessment
Examined skeletal muscles for pathological changes including oxidative damage, protein nitration, myofiber cell death and neuromuscular junction abnormalities
Note: Severe pathology observed in affected mice
View evidence from paper
“Skeletal muscles developed severe pathology involving oxidative damage, protein nitration, myofiber cell death and marked neuromuscular junction (NMJ) abnormalities”
Spinal motor neuron pathology assessment
Analyzed spinal motor neurons for distal axonopathy, ubiquitinated inclusions, and apoptotic-like degeneration involving caspase-3
Note: Motor neurons showed degeneration through apoptotic-like pathway
View evidence from paper
“Spinal MNs developed distal axonopathy and formed ubiquitinated inclusions and degenerated through an apoptotic-like pathway involving capsase-3”