Modelling the dorsal root ganglia using human pluripotent stem cells: A platform to study peripheral neuropathies

Serena Viventi, Mirella Dottori, The International Journal of Biochemistry & Cell Biology, Volume 100, July 2018, Pages 61-68, ISSN 1357-2725, doi:10.1016/j.biocel.2018.05.005.

Sensory neurons of the dorsal root ganglia (DRG) are the primary responders to stimuli inducing feelings of touch, pain, temperature, vibration, pressure and muscle tension. They consist of multiple subpopulations based on their morphology, molecular and functional properties. Our understanding of DRG sensory neurons has been predominantly driven by rodent studies and using transformed cell lines, whereas less is known about human sensory DRG neurons simply because of limited availability of human tissue. Although these previous studies have been fundamental for our understanding of the sensory system, it is imperative to profile human DRG subpopulations as it is becoming evident that human sensory neurons do not share the identical molecular and functional properties found in other species. Furthermore, there are wide range of diseases and disorders that directly/indirectly cause sensory neuronal degeneration or dysfunctionality. Having an in vitro source of human DRG sensory neurons is paramount for studying their development, unique neuronal properties and for accelerating regenerative therapies to treat sensory neuropathies. Here we review the major studies describing generation of DRG sensory neurons from human pluripotent stem cells and fibroblasts and the gaps that need to be addressed for using in vitro-generated human DRG neurons to model human DRG tissue.
There are vast ranges of diseases and conditions, usually progressive, which can affect DRG sensory neurons. The underlying causes of DRG degeneration may be either directly intrinsic to DRG neurons or indirectly associated with other pathologies. Some inherited genetic diseases inducing DRG degeneration include Friedreich’s Ataxia and Charcot Marie Tooth Disease

  Modelling the dorsal root ganglia using human pluripotent stem cells: A platform to study peripheral neuropathies