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Motoneuron Disease

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Neurotrophic Factors

Part of the book series: Handbook of Experimental Pharmacology ((HEP,volume 220))

Abstract

Amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) represent the two major forms of motoneuron disease. In both forms of disease, spinal and bulbar motoneurons become dysfunctional and degenerate. In ALS, cortical motoneurons are also affected, which contributes to the clinical phenotype. The gene defects for most familial forms of ALS and SMA have been discovered and they point to a broad spectrum of disease mechanisms, including defects in RNA processing, pathological protein aggregation, altered apoptotic signaling, and disturbed energy metabolism. Despite the fact that lack of neurotrophic factors or their corresponding receptors are not found as genetic cause of motoneuron disease, signaling pathways initiated by neurotrophic factors for motoneuron survival, axon growth, presynaptic development, and synaptic function are disturbed in ALS and SMA. Better understanding of how neurotrophic factors and downstream signaling pathways interfere with these disease mechanisms could help to develop new therapies for motoneuron disease and other neurodegenerative disorders.

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  1. Institute for Clinical Neurobiology, University of Würzburg, Versbacherstr. 5, 97078, Würzburg, Germany

    M. Sendtner

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  1. Department of Neuroscience, Max-Delbrück-Center for Molecular Medicine, Berlin, Germany

    Gary R. Lewin

  2. Department of Biochemistry, Vanderbilt University School of Medicine, Nashville, Tennessee, USA

    Bruce D. Carter

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Sendtner, M. (2014). Motoneuron Disease. In: Lewin, G., Carter, B. (eds) Neurotrophic Factors. Handbook of Experimental Pharmacology, vol 220. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-45106-5_15

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