TABLE 5.

Zebrafish models of SOD1 pathology.

Study	Method(s)	Axonopathy	MN loss	NMJ abnormalities	Motor deficits	Other phenotypes
Benedetti et al., 2016	SOD1 G93R mutation	Yes – Reduced primary and unbranched axonal length, and increased aberrant branching	Yes	No	Yes – Reduced swim distance and duration	Reduced innervation and muscle atrophy. Increased inflammation and reactive astrogliosis.
Da Costa et al., 2014	TILLING-mediated SOD1 T70I missense mutation	Not reported	Yes	Yes – Reduced colocalization of SV2 and α-bungarotoxin in the interseptal region	Yes – Reduced swim duration and velocity
Lemmens et al., 2007	SOD1 G93A, G37R, and A4V mutations	G93A: Yes – 64.6% of injected. G37R: Yes – 68.8% of injected. A4V: Yes – 73.1% of injected.	Not reported	Not reported	Not reported
Ramesh et al., 2010	BAC-mediated SOD1 G93R mutation	No	Yes	Yes – Reduced colocalization of SV2 and α-bungarotoxin, as well as NMJ volume (in adults)	Yes – Reduced swim endurance (in adults)	Muscle atrophy. Progressive and intermittent paralysis. Increased mortality.
Sakowski et al., 2012	SOD1 G93A mutation	Yes – Reduced axon length and increased branching	Yes	Yes – Loss of intact NMJ (from 20 weeks)	Yes – Reduced swim velocity and progressive reduced swim duration