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. 2020 Aug;30(8):1083–1096. doi: 10.1101/gr.265017.120

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© 2020 Nichterwitz et al.; Published by Cold Spring Harbor Laboratory Press

This article, published in Genome Research, is available under a Creative Commons License (Attribution 4.0 International), as described at http://creativecommons.org/licenses/by/4.0/.

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Figure 5. — Common and cell type–specific disease mechanisms in SMA. Expression changes in key genes between SMA and wild type in CN3/4 (A) and SC motor neurons (B). Colors indicate significance levels. (C) Somatic motor neurons display transcriptional changes caused by the loss of full-length SMN protein that are distinct from red nucleus and vagus (CN10) neurons. For example, prominent changes in expression levels of genes that function in RNA processing are restricted to somatic motor neurons. These neurons are furthermore exposed to cellular stress, including oxidative stress and DNA damage, and DNA repair genes are induced. TRP53- and cell death signaling pathways are activated in all somatic motor neurons independent of their susceptibility to degeneration in SMA. Vulnerable spinal (SC) motor neurons show signs of axon degeneration and axonal transport deficits. Resistant ocular (CN3/4) motor neurons selectively up-regulate the expression of genes that counteract apoptosis and promote cell survival. Increased levels of genes functioning in neurite outgrowth, axon regeneration, and neurotransmission, which support the maintenance of a functional neuromuscular synapse, are also seen in ocular motor neurons in disease.