TABLE 8.

Zebrafish models of C9orf72 pathology.

Study	Method	Axonopathy	MN loss	Motor deficits	Other phenotypes
Ciura et al., 2013	AMO knockdown	Yes – Reduced motor neuron axon length and aberrant branching	Not reported	Yes – Deficient touch-evoked escape response (TEER); reduced swim distance, average velocity and maximum velocity
Lee et al., 2013	8×, 38×, and 72× GGGGCC repeats	Not reported	Not reported	Not reported	Increased apoptotic cell death in 38× and 72× embryos
Ohki et al., 2017	2× or 80× GGGGCC repeats (with or with ATG codon)	No	Not reported	Not reported	Display of toxicity and pericardial edema in 80× embryos
Shaw et al., 2018	89× GGGGCC repeats	Not reported	Yes	Yes – Impaired ability to transition into fast movement. Displayed center avoidance behavior.	Muscle atrophy. Reduced weight gain. Increased mortality.
Swinnen et al., 2018	3×, 4×, 10×, ∼35×, ∼70×, and ∼90× GGGGCC repeats	Yes – Only from ∼35× repeats	Not reported	Not reported