FIGURE 5.

PNS nerves are hypomyelinated in Lpin1^1Hubr rats. A, Nile Red staining of sciatic nerve sections from wild-type (mWT) and Lpin1^fld/fld mice at PND 56, and wild-type (rWT) and Lpin1^1Hubr rats at PND 21 and 90. Nile Red staining demonstrated that Lpin1^fld/fld sciatic nerve specifically accumulates neutral lipids in the perineurium and endoneurium consecutive to demyelination. In the rat, at PND 21, the typical “donut”-like myelin structures present in wild-type sciatic nerve are replaced in Lpin1^1Hubr nerves by “dot”-like structures probably corresponding to the accumulation of neutral and polar lipids in SCs. Lpin1^1Hubr nerves partially recover the donut-like myelin staining at PND 90 suggesting the presence of hypomyelination rather than demyelination. B, relative gene expression analysis of Mpz and Pmp22 (mature SC markers) in sciatic nerve tissue of Lpin1^1Hubr rats as compared with wild-type rats at PND 4, 10, 21 and 90 (n = 3, n = 3, n = 3, and n = 2 per group, respectively, **, p < 0.001). Data are expressed as mean ± S.E.