| First Author | Uschkureit T | Year | 2000 |
| Journal | J Neurosci | Volume | 20 |
| Issue | 14 | Pages | 5225-33 |
| PubMed ID | 10884306 | Mgi Jnum | J:63480 |
| Mgi Id | MGI:1861053 | Doi | 10.1523/JNEUROSCI.20-14-05225.2000 |
| Citation | Uschkureit T, et al. (2000) Early onset of axonal degeneration in double (plp-/-mag-/-) and hypomyelinosis in triple (plp-/-mbp-/-mag-/-) mutant mice. J Neurosci 20(14):5225-33 |
| abstractText | Double (plp-/-mag-/-) and triple (plp-/-mbp-/-mag-/-) null-allelic mouse lines deficient in proteolipid protein (PLP), myelin-associated glycoprotein (MAG), and myelin basic protein (MBP) were generated and characterized genetically, biochemically, and morphologically including their behavioral capacities. The plp-/-mag-/- mutant develops a rapidly progressing axon degeneration in CNS with severe cognitive and motor coordinative deficits but has a normal longevity. CNS axons of the plp-/-mbp-/-mag-/- mouse are hypomyelinated and ensheathed by 'pseudomyelin' with disturbed protein and complex lipid composition. The shiverer trait in the plp-/-mbp-/-mag-/- similar to the plp-/-mbp-/- mutant is significantly ameliorated, and its lifespan is considerably prolonged. The longevity of these dysmyelinosis mouse mutants recommends them as suitable models for the long-term evaluation of stem cell therapeutic strategies. |
