Table 1: Loss-of-function studies for DGC components.

Disrupted genes (animal)Expression level and modification of Schwann cell proteinsNerve pathology and functional deficitsReferences

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
[5561]
Lama2/point mutation of LN domain/CxxC motif (dynmf417 mouse)Normal expression of the mutated laminin 𝛼 2 chainRadial 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, 6365]
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 4Radial 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 mouseSeverely reduced expression of all known laminin isoforms in Schwann cellsRadial sorting defect in spinal roots and peripheral nerves[68]
L-Periaxin (mouse)No expression of L-periaxinDemyelination 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 utrophinDisrupted 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 mouseDeficiency 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 mouseNo expression of 𝛽 1 integrinRadial sorting defect in sciatic nerve
Disrupted BM
[72]
Schwann cell-selective 𝛽 4 integrin null mouseNo expression of 𝛽 4 integrinMyelin folding[54]
Schwann cell-selective 𝛽 4 integrin/Dag1 null mouseNo expression of DG and 𝛽 4 integrinRadial sorting defect in spinal ventral roots
Severe myelin folding
Demyelination
Disrupted BM
[54]

BM: basement membrane, DG: dystroglycan, S-L: Schmidt-Lanterman, NMJ: neuromuscular junction, Sc: Schwann cell.