The “CMT rat”: peripheral neuropathy and dysmyelination caused by transgenic overexpression of PMP22
We have generated a transgenic rat model of Charcot-Marie-Tooth disease type 1A (CMT1A) providing formal proof that this neuropathy can be caused by increased expression of peripheral myelin protein-22 (PMP22). Heterozygous PMP22-transgenic rats develop muscle weakness and gait abnormalities as well...
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| Hauptverfasser: | , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
1999
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| In: |
Annals of the New York Academy of Sciences
Year: 1999, Jahrgang: 883, Pages: 254-261 |
| ISSN: | 1749-6632 |
| DOI: | 10.1111/j.1749-6632.1999.tb08587.x |
| Online-Zugang: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1111/j.1749-6632.1999.tb08587.x Verlag, lizenzpflichtig, Volltext: https://nyaspubs.onlinelibrary.wiley.com/doi/abs/10.1111/j.1749-6632.1999.tb08587.x 
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| Verfasserangaben: | Stephan Niemann, Michael W. Sereda, Moritz Rossner, Helen Stewart, Ueli Suter, Hans-Michael Meinck, Ian R. Griffiths, and Klaus-Armin Nave |
MARC
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| 245 | 1 | 4 | |a The “CMT rat” |b peripheral neuropathy and dysmyelination caused by transgenic overexpression of PMP22 |c Stephan Niemann, Michael W. Sereda, Moritz Rossner, Helen Stewart, Ueli Suter, Hans-Michael Meinck, Ian R. Griffiths, and Klaus-Armin Nave |
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| 520 | |a We have generated a transgenic rat model of Charcot-Marie-Tooth disease type 1A (CMT1A) providing formal proof that this neuropathy can be caused by increased expression of peripheral myelin protein-22 (PMP22). Heterozygous PMP22-transgenic rats develop muscle weakness and gait abnormalities as well as reduced nerve conduction velocities and EMG abnormalities, which closely resemble recordings in patients with CMT1A. Dys- and demyelination, Schwann cell hypertrophy, and “onion bulb” formation are also similar to findings in humans. When bred to homozygosity, transgenic rats completely fail to elaborate myelin, but all myelin-forming Schwann cells segregate with axons in the normal one-to-one ratio. Although arrested at this “promyelin” stage, differentiation proceeds in homozygous rats at the molecular level, as demonstrated by high-level expression of myelin structural genes. Intracellular trafficking of the wild-type protein is not visibly impaired, even when strongly overexpressed, suggesting that PMP22 blocks myelin assembly in a late Golgi/cell membrane compartment of the affected Schwann cell. | ||
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