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
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Materials1
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Protocol Steps
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”