TABLE 2.
Neuronal stress | Electro physiology | NMJ denervation | Cellular changes, insoluble aggregates | Astrocytosis/ Microgliosis/ Mitochondria/ Inclusion/UPS | Gross motor symptoms | |
---|---|---|---|---|---|---|
Human | ? | Prior to onset in adults† | Prior to onset in adults† | Golgi, mitochondria, insoluble aggregates In post-mortem specimens | In post-mortem specimens | 40-50 years |
Mouse (Sod1G93 AHiGur) | ? | 12 dpf* to 6 dpp** (G85R, G93Alow) | ∼30dpp | Golgi changes ∼40-50dpp | ∼70-90dpp | ∼100dpp |
Zebrafish (G93R) | 24 hpf ** (This manuscript) | 96hpf** (This manuscript) | Normal NMJ but reduced staining at 11dpf. 6 months and 1 year Observed denervation in stressed motor neuron (This manuscript) | UPR at 1 month***. | No gross inflammation (unpublished) Motor neuron stress. 6-months (This manuscript) | 1 year |
dpf: days post fertilization; hpf: hours post fertilization; dpp: days postpartum. UPR: Unfolded Protein Response †earliest tested, * earliest tested in organotypic culture; ** Earliest tested in intact spinal cord, *** Personal communication (C.Beattie)