Table 1.

Comparison of transgenic animal models of OPMD

	C. elegans [79]	D. melanogaster [81]	Mouse Hino et al. [87]	Mouse Dion et al. [88]	Mouse Mankodi et al. [93]	Mouse Davies et al. [83]
PABPN1 transgene	Ala13 (human)	Ala17 (bovine)	Ala13 (human)	Ala13 (human)	Ala16 (human)	Ala17 (bovine)
Copies of PABPN1	2 endogenous + transgene	2 endogenous + transgene	2 endogenous + transgene	2 endogenous + transgene	2 endogenous + transgene	2 endogenous + transgene
Promoter driving transgene expression	Muscle-specific Minor myosin heavy chain (Pmyo-3)	Muscle-specific Myosin heavy chain (Mhc-Gal4)	Ubiquitous CMV early enhancer/chic ken beta actin (CAG)	Ubiquitous Putative native PABPN1	Ubiquitous Mifepristone- inducible system	Muscle-specific Human skeletal actin
PABPN1 nuclear aggregates	Yes (weak)	Yes	Yes	Yes (neurons only)	Yes	Yes
Muscles examined for histopathology	Body wall muscle cells	Indirect flight muscles	Limb, eyelid, pharyngeal	Eyelid, soleus	Limb, pharyngeal, diaphragm, paraspinals	Biceps
Muscle histology	Loss of muscle cells	Muscle degeneration Thin, disorganized myofibrils Apoptotic nuclei Loss of mitochondria	Muscle degeneration Increased fibrosis Increased fat	Abnormal increase in regenerated myofibers	Progressive non- necrotic myopathy	Apoptosis Abnormal increase in regenerated myofibers
Phenotype	Abnormal motility: Decreased amplitude of sinusoidal wave Increased body bends	Abnormal wing position: Wings held up Wings held down Dysregulation of ubiquitin-proteasome pathway genes	Muscle weakness Decreased survival Reduced body weight	Muscle weakness Primarily peripheral nerve alterations	Muscle weakness Decreased survival Cardiomyopathy	Progressive muscle weakness Myofiber atrophy Dysregulation of ubiquitin-proteasome pathway genes
Age of onset*	5 days (~25-day lifespan)	2 days (~25-day lifespan)	3 months (juvenile)	12 months (adult)	10–12 weeks after induction	6 months (adult)

^*Age at which functional muscle deficits were first documented.