Table 1.

ECM Schwann cell Phenotypes

Mouse	Protein levels	Basal Lamina Appearance	Radial Sorting Defect	Myelin phenotype				Remyelination	Schwann cell development	Nodal Architecture	References
				Thinner	Thicker	Outfoldings	Stability
dy	Decreased levels of laminin α2; increased levels of laminin α4	Disrupted	Yes (esp. in proximal region of peripheral nerve); rare polyaxonal myelination	Yes	No	No	N.D.	Normal remyelination after nerve crush	Impaired SC maturation; immature SCs extend processes within axon bundles	Wider nodes; partial paranodal abnormality	Stirling 1975, Bradley 1977, Jaros and Bradley 1979, Bray 1983; Okada et al 1977
dy2J	Slightly reduced levels of truncated laminin α2 mutant; increased levels of laminin α4 in nerves; aberrant expression of laminin α1 in nerves	Disrupted; thin lamina densa	Yes (esp. in proximal region of peripheral nerve); rare polyaxonal myelination	N.D.	No	No	N.D.		Decreased SC numbers in spinal root; immature SCs extend processes within axon bundles	Wider nodes; abnormal Nav clustering; disorganized SC microvilli	Stirling 1975, Bradley and Jenkison 1973, Bray and Aguayo 1975, Okada 1977, Bradley and Jaros 1979, Patton 1997, Yang 2005; Occhi 2005
dy3K	Lack of laminin α2; increased levels of laminin α4	Disrupted	Yes in spinal roots and in proximal peripheral nerves	Yes, with some sorted, but unmyelinated axons	No	No	Normal	N.D.	N.D.	Decreased nodal gap lengh	Nakagawa 2001
nmf417	Expression of C79R laminin α2 mutant at ‘normal’ levels	Normal; thicker lamina densa (rare)	Yes (esp. in the proximal region of peripheral nerve)	No	No	No	normal	N.D.	Immature SCs do not extend processes within axon bundles	N.D.	Patton 2008
Laminin α4 KO	Lack of laminin α4	Outfoldings	Yes, followed by partial recovery; polyaxonal myelination; defect esp. in distal region of peripheral nerve	Yes	No	No	Normal	N.D.	Decreased SC numbers	N.D.	Yang 2005; Rasi 2010
dy2J/Laminin α4 double mutant	Slightly reduced levels of truncated laminin α2 mutant; Lack of laminin α4	Deficient in pre- and pro-myelinating SCs	Yes, severe throughout peripheral nerve. Normal sorting in spinal roots	Yes	No	No	Normal	N.D.	Decreased SC proliferation and numbers	N.D.	Yang 2005
Laminin α2/〈4 double knockout	Lack of laminin α2 and α4	N.D.	Yes, complete lack of sorting in both nerve and root (analysis ends at P11 due to early death).	Yes	No	No	Normal	N.D.	Decreased SC proliferation and numbers	N.D.	Yang 2005
Nervous system-specific Laminin γ1 knockout (CaMKII-Cre)	Lack of laminin γ1 in a subpopulation of SCs	Disrupted	Yes, severe	No	No	No	normal	N.D. (Impaired axonal regeneration after nerve crush)	Impaired SC differentiation; normal SC proliferation and apoptosis	N.D.	Chen and Strickland 2003
SC-specific Laminin γ1 knockout (P0-cre)	Lack of laminin γ1 in SCs; lack of all SC laminins: laminins-111, -211, -411, and -511	Disrupted	Yes, severe	No	No	No	normal	N.D.	Impaired SC differentiation; decreased proliferation; Increased apoptosis	N.D.	Yu 2005
LMα1Tg/dy3K	Lack of laminin α2; increased levels of laminin α1	Normal	No	No	No	No	Normal	N.D.	normal	normal	Gawlik 2006
δE3LMα1/dy3K	Lack of laminin α2; expression of LG1 domain of laminin α1	Disrupted	Yes (esp. in the proximal region of peripheral nerve); polyaxonal myelination	No	Yes	Yes	Degeneration (onion bulbs)	N.D.	Detachment of myelinating SCs from axons; pre-apoptotic phenotype; abnormal	N.D.	Gawlik 2010
Collagen XV knockout	Lack of collagen XV	Normal; rarely protrusions	No; polyaxonal myelination	Yes	No	No	Normal	N.D.	N.D.	N.D.	Rasi 2010
Collagen XV/laminin α4 double knockout	Lack of collagen XV and laminin α4	Normal; slightly increased thickness	Yes; no recovery; polyaxonal myelination	Yes	No	No	Normal	N.D.	N.D.	N.D.	Rasi 2010