Publication: The Gdap1 knockout mouse mechanistically links redox control to Charcot-Marie-Tooth disease
The Gdap1 knockout mouse mechanistically links redox control to Charcot-Marie-Tooth disease
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Niemann, A., Huber, N., Wagner, K. M., Somandin, C., Horn, M., Lebrun-Julien, F., Angst, B., Pereira, J. A., Halfter, H., Welzl, H., Feltri, M. L., Wrabetz, L., Young, P., Wessig, C., Toyka, K. V., & Suter, U. (2014). The Gdap1 knockout mouse mechanistically links redox control to Charcot-Marie-Tooth disease. Brain : A Journal of Neurology, 137(Pt 3), 668–682. https://doi.org/10.1093/brain/awt371
Abstract
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Abstract
The ganglioside-induced differentiation-associated protein 1 (GDAP1) is a mitochondrial fission factor and mutations in GDAP1 cause Charcot-Marie-Tooth disease. We found that Gdap1 knockout mice (Gdap1(-/-)), mimicking genetic alterations of patients suffering from severe forms of Charcot-Marie-Tooth disease, develop an age-related, hypomyelinating peripheral neuropathy. Ablation of Gdap1 expression in Schwann cells recapitulates this phenotype. Additionally, intra-axonal mitochondria of peripheral neurons are larger in Gdap1(-/-) mic
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- Niemann, Axel
- Huber, Nina
- Wagner, Konstanze M
- Somandin, Christian
- Horn, Michael
- Lebrun-Julien, Frédéric
- Angst, Brigitte
- Pereira, Jorge A
- Halfter, Hartmut
- Welzl, Hans
- Feltri, M Laura
- Wrabetz, Lawrence
- Young, Peter
- Wessig, Carsten
- Toyka, Klaus V
- Suter, Ueli
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Niemann, A., Huber, N., Wagner, K. M., Somandin, C., Horn, M., Lebrun-Julien, F., Angst, B., Pereira, J. A., Halfter, H., Welzl, H., Feltri, M. L., Wrabetz, L., Young, P., Wessig, C., Toyka, K. V., & Suter, U. (2014). The Gdap1 knockout mouse mechanistically links redox control to Charcot-Marie-Tooth disease. Brain : A Journal of Neurology, 137(Pt 3), 668–682. https://doi.org/10.1093/brain/awt371
