Because several of the behavioral defects were consistent with hyperactivity, we examined the underlying neuronal defects and found that sensory neurons and motoneurons appeared normal.
In Drosophila, overexpression of autophagy‐related genes in neurons enhances longevity, while their repression causes neuronal defects and shortening of lifespan (Simonsen et al., 2008).
Third, transient treatment of MEKi exhibited a robust long-term rescue of glial and neuronal defects in the cerebella of G-responders in which neurons and glia were still deficient for Nf1 after the treatment.
Homozygous foggy m806 fish look relatively normal but have neuronal defects that ultimately prove lethal, including a deficit of dopamine-containing neurons and corresponding excess of serotonin-containing neurons 31.
Many ciliopathies have neuronal defects.
Depletion of Drosophila or zebrafish ATL results in neuronal defects (Lee et al., 2008; Lee et al., 2009; Fassier et al., 2010).
Using such Tauopathy models, compounds with known anti-aggregation activity like methylene blue, were shown to effectively ameliorate the worms' motility and neuronal defects [36].
Neuronal defects in RSTS can be attributed to genetic re-arrangements in CREBBP, which has been implicated in synaptic plasticity and long-term memory.
Indeed, a focused screen of pharmacological compounds that ameliorated the dnj-14 lifespan and neuronal defects identified the polyphenolic molecule resveratrol, which has been shown to be neuroprotective in a range of animal neurodegeneration models [71].
Toxicogenetic ablation of CINs in young mice reproduced these behavioral and neuronal defects, suggesting that age-related deficits in PF-to-CIN PF-to-CIN PF-to-CIN ability ofunctionindividuals to reducee conflictheetween ability, likely coftributing tolderaindividualsadaptove goal-diresolveaconflictd executive control in aging.
This includes inherent neuronal defects resulting in neuromuscular dysfunction.
