4.5 Review

Long non-coding RNAs in motor neuron development and disease

Journal

JOURNAL OF NEUROCHEMISTRY
Volume 156, Issue 6, Pages 777-801

Publisher

WILEY
DOI: 10.1111/jnc.15198

Keywords

long non-coding RNA; circular RNA; motor neuron development; spinal muscular atrophy; amyotrophic lateral sclerosis

Funding

  1. ALS Stichting Nederland
  2. Dutch Epilepsiefonds [WAR 18-05]
  3. [721890]

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Long non-coding RNAs play crucial roles in the expression and development of motor neurons in the nervous system, and disruptions in their effects may contribute to motor neuron diseases. Research on the expression and functions of lncRNAs in motor neurons is shedding light on potential therapeutic targets for various motor neuron diseases.
Long non-coding RNAs (lncRNAs) are RNAs that exceed 200 nucleotides in length and that are not translated into proteins. Thousands of lncRNAs have been identified with functions in processes such as transcription and translation regulation, RNA processing, and RNA and protein sponging. LncRNAs show prominent expression in the nervous system and have been implicated in neural development, function and disease. Recent work has begun to report on the expression and roles of lncRNAs in motor neurons (MNs). The cell bodies of MNs are located in cortex, brainstem or spinal cord and their axons project into the brainstem, spinal cord or towards peripheral muscles, thereby controlling important functions such as movement, breathing and swallowing. Degeneration of MNs is a pathological hallmark of diseases such as amyotrophic lateral sclerosis and spinal muscular atrophy. LncRNAs influence several aspects of MN development and disruptions in these lncRNA-mediated effects are proposed to contribute to the pathogenic mechanisms underlying MN diseases (MNDs). Accumulating evidence suggests that lncRNAs may comprise valuable therapeutic targets for different MNDs. In this review, we discuss the role of lncRNAs (including circular RNAs [circRNAs]) in the development of MNs, discuss how lncRNAs may contribute to MNDs and provide directions for future research.

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