Table 1.

Currently existing mouse models to study ERMS [95].

Genes/Targeted Pathways	Genetic Modification in Mouse	FN-RMS	References
RAS	Expression of KRAS G12D expression (±background of heterozygous or homozygous p53)	UPS with myogenic features	[96]
FGFR4	Murine myoblasts expressing FGFR4V550E	RMS	[97]
P53	Knock-out of p53 in C57BL/6	RMS	[90]
	Trp53/FOS double knock-out in 129Sv X C57BL/6	ERMS	[98]
	Constitutively expressed HER2/neu in Balb/c with p53+/− via MMTV promotor	ERMS	[99]
	KRAS G12V conditional expression in adult Balb/c, expressed from Rag2 promotor	ERMS	[100]
	Transgenic KRASG12V expression with concurrent knock-out of p53/gain of p53R172H mutant in C57B16J/S129 mice	PRMS	[101]
HGF	HGF/SF Overexpression of HGF in albino FVB/N mice	PRMS with lung metastases	[91,92]
Sonic hedgehog	Inactivation of Ptch mutations	RMS	[102]