Table 1.

Major Muscular Dystrophies (MD) modelled in Drosophila and zebrafish.

Disease	Mutated Gene	Animal Model		Mutation Type in Animal Models		Shared Symptoms with Patients	Ref.
		D	Z	Drosophila	Zebrafish
DMD/BMD	Dystrophin	✓	✓	Dys deletion mutants	Nonsense mutation in dystrophin gene	Age-dependent muscle degeneration/Loss of muscle integrity	[33,55]
					Splice mutation in dystrophin gene		[60,61,62]
LGMD	Lamin A/C Sarcoglycan Dysferlin POMT1	✓	✗	Partial/null mutations in the Drosophila δ-sarcoglycan locus	✗	Reduced lifespan & mobility in aged flies	[56]
DM1	DMPK	✓	✓	Expression of CTG repeats in adult/larval muscle	MBNL gene knockdown	Myotonia/Muscle defects Splicing defects Foci formation	[35,36]
					Injection of CUG repeat-containing mRNA		[37,38,68]
OPMD	PABPN1	✓	✗	Human PABPN1-17ala expressed in adult muscle	✗	Progressive muscle degeneration Nuclear inclusions	[57]
EDMD	Emerin Lamin A/C	✓	✗	Transgenic flies expressing a mutant form of Lamin-C (lacking the first 42 AA)	✗	Muscle defects Early death	[58,59]
CMD	POMT1 Fukutin Laminin α2	✓	✓	Mutants for POMT1 & POMT2 Drosophila orthologs	Point mutation in laminin α2 gene	Shortened lifespan Age-dependent severity of muscle phenotypes/Disorder of primary motor neurons innervation	[39,40]

Notes: Abbreviations: D Drosophila, Z Zebrafish, DMD Duchenne MD, BMD Becker MD, LGMD Limb-Girdle MD, DM1 Myotonic Dystrophy Type 1, OPMD Oculo-Pharyngeal MD, EDMD Emery-Dreifuss MD, CMD Congenital MD. More comprehensive tables of additional models can be found in other recent reviews such as [32,63].