Table 1.

Existing animal models for aaRS-induced CMT through dominant mutations

Gene/CMT subtype	Animal	Mutation	Genetic manipulation	Phenotype severity	Refs.
GARS/CMT2D	Mouse	C157R	Knockin	P234KY > C157R	61
		P234KY	Knockin		60
	Drosophila	E71G	Transgenic overexpression	P234KY > G240R > G526R > E71G	41
		P234KY	Transgenic overexpression		64
		G240R	Transgenic overexpression		41, 64
		G526R	Transgenic overexpression		41
YARS/DI-CMTC	Drosophila	G41R	Transgenic overexpression	E196K > G41R > Δ153–156	40
		Δ153–156	Transgenic overexpression		40
		E196K	Transgenic overexpression		40
AARS/CMT2N	Zebrafish	R326W	mRNA injection of embryos	Varies among experiments	27
		E337K	mRNA injection of embryos		27
		S627L	mRNA injection of embryos		27
HARS/CMT2W	C. elegans	R137Q	Transgenic overexpression	Not reported	44
		D364Y	Transgenic overexpression		42