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Disorder | Species | Model | Ganglioside alterations | Clinical signs | References |
|
AD | Human | Early-onset AD | Total ganglioside | Lower ganglioside level in frontal white and gray matter. | [14] |
AD | Human | Late-onset AD | Total ganglioside | Lower ganglioside level in temporal cortex, hippocampus, and frontal white matter of patients. | [14] |
AD | Human | AD | GD1b, GT1b | Reduction ganglioside in hippocampal gray matter. | [15] |
AD | Mouse | 1XFAD/GM2S-/- | GM3, GD3 | Exacerbation of Alzheimer’s pathology | [9] |
AD | Mouse | 2XFAD/GD3S-/- | GM1, GD1a, GM3 | Amelioration of disease pathology | [16] |
AD | Mouse | 5XFAD/GM3S-/- | Lack of major gangliosides | Lower Aβ deposition and neuroinflammation | [17] |
AD | Mouse | 5xFAD//Ugcgf/f//Thy1-CreERT2//EYFP | GM3, GM2, GT1b | Improve memory and loss of dendritic spines | [18] |
PD | Human | PD | GM1 | Reduction of GM1 in occipital cortex | [19] |
PD | Human | PD | GM1, GD1a | Reduction of gangliosides in heart, colon, and skin tissues. | [20] |
PD | Mouse | B4GALNT1-/- | GM3, GD3 | Accumulation of αSyn deposition in SN. | [21] |
PD | Mouse | B4GALNT1+/- | GM1, GD1a, GD1b | PD-like motor functions and αSyn accumulation | [11] |
HD | Human | HD | GM1 | Reduction of GM1 in fibroblast. | [10] |
HD | Human | HD | GM1 | Increased GM1 in the cerebellum. | [22] |
HD | Human | HD | ST8Sia3 and B4Galnt1 | Decreased expression of ganglioside synthesis enzymes in the brain. | [23] |
HD | Mouse | R6/1 | GM1 | Molecular, behavioral, and motor disturbances | [22] |
HD | Mouse | YAC128 | GM1, GD1a, GT1b | Neurodegeneration and motor function abnormalities | [10] |
ALS | Human | ALS | GM2 | Increased GM2 in the motor cortex | [24] |
ALS | Human | ALS | GM3 and GM1 | Increased GM3 and GM1 in spinal cord. | [13] |
ALS | Mouse | SOD1G93A | GM3 | Gait impairment and reduced motor function | [13] |
ALS | Mouse | SOD1G86R | GM1a, GM2, GM3 | Spinal motor neuron degeneration, progressive skeletal muscle weakness | [25] |
HSP | Mouse | B4GALNT1-/- | GM3, GD3 | Deficiency of hippocampal plasticity, axonal degeneration | [26, 27] |
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