Table 2.

Overview of animal models referred to in this review

Species	Endogenous SMN orthologue	Modelling strategy and/or genotype		References
Caenorhabditis elegans	CeSMN	Knockdown of expression through RNAi		[209]
Drosophila melanogaster	DmSMN	Point mutations or transposon insertions for knockout or knockdown studies		[210, 211]
Danio rerio	Smn	Knockdown of expression through antisense oligonucleotides		[47]
Mus musculus	Smn
Smn knockout		Smn − /−	Smn null mutation by targeted insertion of β-galactosidase in Smn exon 2A	[2]
Taiwanese mice		Smn H7/H7 ; SMN2Hung tg/−	Two copies of the SMN2 transgene, Smn exon 7 is replaced with hypoxanthine phosphoribosyl transferase (HPRT) but transcripts without exon 7 are produced.	[43]
SMNΔ7		Smn 2A/2A ; SMN2 tg/tg ; SMN∆7 tg/tg	One copy of the SMN2 transgene and one SMNΔ7 transgene on Smn null background	[45]
Smn2B		Smn 2B/−	Mutation within the splicing enhancer of Smn exon 7 producing SMN2-like transcripts and reduced FL-SMN protein	[212]
Burghes severe model		Smn−/−; SMN2tg/tg	Smn null mutation by target replace of β-galactosidase in Smn exon 2A; with one copy of SMN2	[44]

For a comprehensive review of animal models of SMA, see Edens et al. [51]