Table 1.
Disease | Cell type | Phenotype | References |
---|---|---|---|
Alzheimer’s disease | Neurons | Increased deposition of pathological markers amyloid-β and phosphorylated Tau protein Activated glycogen synthase kinase-3β Endosomal abnormalities |
58, 125 |
Frontotemporal dementia | Motor and forebrain neurons | Impaired axonal transport C9ORF72 repeat region containing RNA foci Altered gene expression Diminished neuronal firing capacity |
37, 72 |
Huntington’s disease | Neural precursor cells, forebrain and striatal neurons, astrocytes | Reduced survival Altered gene expression Altered cytoskeleton, adhesion, energetics and neurite outgrowth Reduced neuronal firing capacity Increased susceptibility to stressors Cytoplasmic, electron clear vacuoles in astrocytes |
59, 65, 126 |
Motor neuron diseases: amyotrophic lateral sclerosis Charcot-Marie-Tooth disease giant axonal neuropathy hereditary spastic paraplegia myotonic dystrophy type I spinal-bulbar muscular atrophy spinal muscular atrophy spinal muscular atrophy with respiratory distress type 1 | Motor neurons, astrocytes | Reduced survival Cytoplasmic and nuclear protein aggregation Altered gene expression Neurite degeneration/abnormal growth/decreased complexity Increased oxidative stress and susceptibility to stressors, including PI3K inhibition Altered subcellular axonal transport Activation of endoplasmic reticulum stress and unfolded protein response pathways C9ORF72 repeat region containing RNA foci Intrinsic membrane hyperexicitability and diminished firing capacity Noncell autonomous toxicity from disease-bearing astrocytes |
34, 62, 64, 66, 67, 69, 86, 127–134 |
Parkinson’s disease | Dopaminergic neurons | Reduced survival Increased susceptibility to stressors Mitochondrial dysfunction Elevated disease-associated α-synuclein protein Reduced glucocerebrosidase enzymatic activity Reduced synthesis and release of dopamine Increased monoamine oxidase B expression Impaired intrinsic network activity |
41, 60, 61, 94, 97 |