Abstract
Two crucial questions in neuroscience are how neurons establish individual identity in the developing nervous system and why only specific neuron subtypes are vulnerable to neurodegenerative diseases. In the central nervous system, spinal motor neurons serve as one of the best-characterized cell types for addressing these two questions. In this review, we dissect these questions by evaluating the emerging role of regulatory microRNAs in motor neuron generation in developing embryos and their potential contributions to neurodegenerative diseases such as spinal muscular atrophy (SMA). Given recent promising results from novel microRNA-based medicines, we discuss the potential applications of microRNAs for clinical assessments of SMA disease progression and treatment.
Highlights
How neurons establish individual identity in the developing nervous system and why only specific neuron subtypes are susceptible to degeneration in neurodegenerative disease are two challenging unanswered questions in neuroscience
Deletion of the enzyme Dicer from all motor neurons (MNs) using Olig2-Cre (Dicer MND ) revealed a preferential loss of many limb- and sympathetic gangliainnervating spinal MNs (Chen and Wichterle, 2012). This disruption led to defects in motor pool identity specification, yet the miRNA candidates to maintain motor pool identity have yet to be unveiled (Figure 3D). These results indicate that miRNAs are an integral part of the genetic program controlling MN survival and acquisition of subtype-specific properties
Dysregulated expression in MNs differentiated from ESCs
Summary
How neurons establish individual identity in the developing nervous system and why only specific neuron subtypes are susceptible to degeneration in neurodegenerative disease are two challenging unanswered questions in neuroscience. Answers to these questions are crucial to establishing the patterns of connectivity between neuronal types and their selective targets. Deciphering neuronal subtype is relatively straightforward in Caenorhabditis elegans, as each neuronal lineage and its position, pattern of connectivity, molecular profile, and function have all been well characterized (Hobert, 2008). The complex array of neurons in the vertebrate nervous system (e.g., 1011 in the human brain) renders it rather more challenging to define a neuronal type. Compared to the complexity of a brain, a galaxy is just an inert lump’
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
