Disrupted genes (animal) Expression level and modification of Schwann cell proteins Nerve pathology and functional deficits References Schwann cell-selective Dag1 null mouse (P0-DG null mouse) No expression of DG Reduced expressions of other DGC components except periaxin, laminin-2 Mild radial sorting defect in spinal roots Myelin folding/polyaxonal myelination Mild loss of myelinated axons in sciatic nerve Intact BM Lower conduction Disruption of nodes Disrupted Cajal bands Shortening of internodes [25 , 52 , 54 ] Unknown (dy mouse) No expression of laminin
𝛼
2 Upregulation of laminin
𝛼
4 Radial sorting defect in nerve roots and cranial nerves Mild radial sorting defect in peripheral nerves Disrupted BM Shortening of internodes Lower conduction Widened nodes of Ranvier [55 –61 ] Lama2/point mutation of LN domain/CxxC motif (dynmf417 mouse) Normal expression of the mutated laminin
𝛼
2 chain Radial sorting defect in nerve roots Intact BM [62 ] Lama2/splicing skip of exon2 (dy2J mouse) Normal expression of truncated laminin
𝛼
2 chain (domain IV deleted) Upregulation of laminin
𝛼
4 Radial sorting defect in nerve roots and cranial nerves Mild radial sorting defect in peripheral nerves Multiple axons myelinated by one Sc Disrupted BM Disrupted Cajal bands Shortening of internodes [52 , 55 , 57 , 60 , 63 –65 ] Lama2 (dy3k mouse) No expression of laminin
𝛼
2 Upregulation of laminin
𝛼
4 A few solitary unmyelinated axons Thin myelin sheath Decreased nodal gap Lower conduction Disrupted BM [66 ] Lama4 (mouse) No expression of laminin
𝛼
4 Radial sorting defect in peripheral nerves Polyaxonal myelination Intact BM [67 ] Lama2/Lama4 (dy2J/
𝛼
4 null mouse) Normal expression of truncated laminin
𝛼
2 chain No expression of laminin
𝛼
4 Severe radial sorting defect of peripheral nerves Mild radial sorting defect in spinal roots Disrupted BM [67 ] Schwann cell selective Lamg1-null mouse Severely reduced expression of all known laminin isoforms in Schwann cells Radial sorting defect in spinal roots and peripheral nerves [68 ] L-Periaxin (mouse) No expression of L-periaxin Demyelination in sciatic nerve Myelin folding Disrupted S-L incisure Lower conduction Impaired regeneration Hyperalgesia, allodynia Disrupted Cajal bands Shortening of internodes [51 , 69 ] Utrophin (mouse) No expression of utrophin Disrupted Cajal bands Shortening of internodes [52 ] Sarcoglycans (BIO14.6 hamster) No expression of sarcoglycans Reduced expression of
𝛼
-DG and Dp116 Myelin folding Disrupted S-L incisure [50 ] Large null mouse (enr, myd) Very low expression of Large Hypoglycosylation of
𝛼
-DG Radial sorting defect in sciatic nerve and spinal roots Intact BM Lower conduction Disrupted Cajal bands Impaired regeneration [52 , 70 ] Fukutin chimera mouse Deficiency of fukutin Hypoglycosylation of
𝛼
-DG Radial sorting defect in sciatic nerve and spinal roots Loss of myelinated axons in nerve roots and sciatic nerve at P15–30 Multiple axons myelinated by one Sc Intact BM Disruption of NMJ [71 ] Schwann cell-selective
𝛽
1 integrin null mouse No expression of
𝛽
1 integrin Radial sorting defect in sciatic nerve Disrupted BM [72 ] Schwann cell-selective
𝛽
4 integrin null mouse No expression of
𝛽
4 integrin Myelin folding [54 ] Schwann cell-selective
𝛽
4 integrin/Dag1 null mouse No expression of DG and
𝛽
4 integrin Radial sorting defect in spinal ventral roots Severe myelin folding Demyelination Disrupted BM [54 ]