Abstract
Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder characterized by progressive paralysis resulting from the death of upper and lower motor neurons. There is currently no effective pharmacological treatment for ALS, and the two approved drugs riluzole and edaravone have limited effects on the symptoms and only slightly prolong the life of patients. Therefore, the development of effective therapeutic strategies is of paramount importance. In this study, we investigated whether Miyako Island Bidens pilosa (MBP) can alleviate the neurological deterioration observed in a superoxide dismutase-1 G93A mutant transgenic mouse (G93A mouse) model of ALS. We orally administered 2 g/kg/day of MBP to G93A mice at the onset of symptoms of neurodegeneration (15 weeks old) until death. Treatment with MBP markedly prolonged the life of ALS model mice by approximately 20 days compared to that of vehicle-treated ALS model mice and significantly improved motor performance. MBP treatment prevented the reduction in SMI32 expression, a neuronal marker protein, and attenuated astrocyte (detected by GFAP) and microglia (detected by Iba-1) activation in the spinal cord of G93A mice at the end stage of the disease (18 weeks old). Our results indicate that MBP administered after the onset of ALS symptoms suppressed the inflammatory activation of microglia and astrocytes in the spinal cord of the G93A ALS model mice, thus improving their quality of life. MBP may be a potential therapeutic agent for ALS.
Highlights
Amyotrophic lateral sclerosis (ALS), known as Lou Gehrig’s disease, is a fatal neurodegenerative disease characterized by progressive paralysis due to motor neuron degeneration
To determine whether the therapeutic potential of Miyako Island Bidens pilosa (MBP) was attributable to the suppression of spinal motor neuron degeneration, we evaluated the number of motor neurons in the spinal cord
We show that these improvements were associated with a reduction in reactive astrocytes and activated microglial cells and delayed motor neuron loss in the spinal cord
Summary
Amyotrophic lateral sclerosis (ALS), known as Lou Gehrig’s disease, is a fatal neurodegenerative disease characterized by progressive paralysis due to motor neuron degeneration. Most ALS cases are sporadic, and the cause of sporadic ALS remains largely unknown. Familial ALS (fALS) accounts for the remaining 5 to 10 percent of all ALS cases, and only 20% of fALS cases are linked to a mutation in the gene encoding copper-zinc superoxide dismutase (SOD1) [1]. Several transgenic mouse models that carry the mutations found in fALS patients have been generated. The most widely used model is a transgenic mouse that overexpresses a human SOD1 transgene with a pathogenic glycine to alanine substitution at the 93rd codon (SOD1G93A). Overexpression of the mutant SOD1G93A gene in transgenic mice (G93A mice) results in a progressive paralytic disease in which the clinical features resemble that of ALS in humans [2]. Many new ALS-causing gene defects have been identified, including mutations in the gene encoding fused in sarcoma (FUS), TAR DNA-binding protein (TARDBP), optineurin
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