Table 3.

Phenotypic aspects of wobbler mice compared to sALS and fALS

	Effects, cellular effects/affected processes		Wobbler/ref		ALS/ref
Effects on the organism	Motor defects	+	(Duchen and Strich 1968)	+	(Bruijn et al. 2004)a
	Tremor, hyperreflexia, spasticity	+	(Duchen and Strich 1968)	+	(Bruijn et al. 2004)a
	Muscle weakness, cramps	+	(Duchen and Strich 1968)	+	(Bruijn et al. 2004)a
	Cognitive defects, frontotemporal dementia	–	Not tested	±	In some cases (Achi and Rudnicki 2012)a
	Death due to respiratory failure	+	(Duchen and Strich 1968)	+	(Bruijn et al. 2004)a
Effects on cells	Degeneration of upper and lower motor neurons	+	(Duchen and Strich 1968)	+	(Ferraiuolo et al. 2011)a
	Astrogliosis	+	(Duchen and Strich 1968)	+	(Ferraiuolo et al. 2011)a
	Microgliosis	+	(Duchen and Strich 1968)	+	(Ferraiuolo et al. 2011)a
	Muscle atrophy	+	(Duchen and Strich 1968)	+	(Ferraiuolo et al. 2011)a
	Hyperexcitability, decreased GABAergic inhibition	+	(Nieto-Gonzalez et al. 2011)	+	(Vucic et al. 2008)
	Spermatogenesis defect	+	(Heimann et al. 1991)	–	Not reported
Effects in motor neurons	Vesicle traffic defects	+	(Palmisano et al. 2011)	+	(Ferraiuolo et al. 2011)a
	Enlarged endosomes vacuolization	+	(Palmisano et al. 2011)	+	(Palmisano et al. 2011)
	Impaired axonal transport	+	(Mitsumoto et al. 1990)	+	(Williamson and Cleveland 1999)
	Protein missorting	+	(Perez-Victoria et al. 2008)	+	(Yang et al. 2001; Nishimura et al. 2004)
	Ubiquitin-positive protein aggregates	+	(Dennis and Citron 2009)	+	(Neumann et al. 2006)
	TDP-43-positive protein aggregates	+	(Dennis and Citron 2009)	+	(Neumann et al. 2006)
	Neurofilament aggregations (perinuclear)	+	(Pernas-Alonso et al. 2001)	+	(Hirano et al. 1984)
	Mitochondrial dysfunction	+	(Santoro et al. 2004)	+	(Wiedemann et al. 2002)
	Cortical hyperexcitability/excitotoxicity	+	(Nieto-Gonzalez et al. 2011)	+	(Vucic et al. 2008)

^aEffects, which were several times reported, but recently reviewed in