TABLE 1.

Summary of available LAMA2-CMD mouse models.

Mouse	Mutation	Laminin α2 expression	Phenotype#	Time of death	References
dy/dy	Spontaneous, unknown	Reduced expression of normal sized laminin α2	Moderate muscular dystrophy Peripheral neuropathy	Before 6 months of age	Sunada et al., 1994; Xu et al., 1994a
dy2J/dy2	Spontaneous splice site mutation in LN domain	Slightly reduced expression of truncated laminin α2 missing LN domain	Mild muscular dystrophy Peripheral neuropathy	After 6 months of age	Xu et al., 1994b; Sunada et al., 1995
dy6J/dy6J	Spontaneous, unknown	Unknown	Moderate (?) muscular dystrophy Peripheral neuropathy	Before 6 months of age	https://www.jax.org/strain/003589
dy7J/dy7J	ENU-induced missense mutation in LN domain	Normal levels of normal sized laminin α2	Mild muscular dystrophy Peripheral neuropathy	After 6 months of age	Patton et al., 2008
dyW/dyW	Knock-out	Severely reduced expression of truncated laminin α2 missing LN domain	Severe muscular dystrophy Peripheral neuropathy	5–12 weeks of age	Kuang et al., 1998b; Willmann et al., 2017
dy3K/dy3K	Knock-out	Complete deficiency	Very severe muscular dystrophy Peripheral neuropathy	3 weeks of age	Miyagoe et al., 1997
dy8J/dy8J (extinct)	Spontaneous, unknown	Unknown	Severe (?) muscular dystrophy Peripheral neuropathy	3–4 weeks of age	https://www.jax.org/strain/009692
dyPas/dyPas (extinct)	Spontaneous, retrotransposal insertion between exon 34 and 35	Complete deficiency	Severe muscular dystrophy Peripheral neuropathy	Before 13 weeks of age	Besse et al., 2003

The symbol “#” denotes description of muscular dystrophy severity (mild, moderate, severe, and very severe). The severity is based on age of onset, histopathological severity and time of death. The symbol “?” denotes characterization of mice remains to be published. Description of severity is based on information on https://www.jax.org/.