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Motor neuron pathology was absent.
Whatever the exact contribution of muscle mSOD1 to mitochondrial dysfunction in these mice, the elucidation of the mechanisms underlying motor neuron pathology in MCK-UCP1 mice shed light on the early pathological events of ALS and might then open avenues for potential therapeutic strategies in ALS and other motor neuron disorders.
Hall, C. E. et al. Progressive motor neuron pathology and the role of astrocytes in a human stem cell model of VCP-related ALS.
Spasticity is a common sequela of upper motor neuron pathology, such as cerebrovascular diseases and cerebral palsy.
Criteria for assessing upper motor neuron pathology in lower motor neuron disease (LMND) still remain a major issue in clinical diagnosis.
To uncover targets directly responsible for motor neuron pathology in SBMA which are altered prior to the onset of symptoms, rather than dysregulated as a result of the disease, we undertook a transcriptomic analysis of cultured embryonic motor neurons of AR100 SBMA mice.
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A broad differential diagnoses at this point included peripheral nerve entrapment, peripheral neuropathy, motor neuron pathologies, and muscular disorders.
Taken together, the neuron-specific overexpression of N88S/S90L mutant Seipin leads to slowly progressive upper and lower motor neuron pathologies reminiscent of phenotypes in seipinopathy patients.
Progressive degeneration of α-motor neurons is a pathological hallmark of SMA (Lunn & Wang, 2008), and indeed, we observed motor-neuron pathology in ICV-injected mice.
ICV injection of ASO-20-37 ASO-20-37 ASO-20-37on pathology, and elicitedly when combined with IP injection, it led to NMJ pathology, as well as overt motor-function deficits.
Peripheral neuropathies are sensory- and motor-neuron pathologies caused by genetic defects or stress (Nave, 2010; Cashman and Hoke, 2015).
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