FIGURE 1.
Overview of the sensory-motor system and pathologies observed in SMA. Schematic (A) and immunohistochemical (B) representation of the sensory-motor system, focusing on structures, cell types, and subcellular compartments that have been implicated in SMA pathogenesis. In (A), the text in green indicates the primary pathological changes occurring in specific cell types or in specific subcellular compartments. In (B), immunofluorescence was used to label parvalbumin-expressing cells and projections (proprioceptive neurons; red), neuronal cell bodies (ChAT for motor neurons, beta-III-tubulin for DRG cell bodies; green), and nuclei (DAPI; blue). Note that only a subgroup of DRG cell bodies express parvalbumin and that other markers such as NF200+ (mechano- and proprioceptive) and peripherin+ (nociceptive) can also be used to identify other types of sensory neurons. Scale bars: 100 μm (spinal cord and DRG); 20 μm (motor neuron). DRG, dorsal root ganglion; ChAT, acetylcholine transferase; DAPI, 4,6-diamidino-2-phenylindole; vGlut1, vesicular glutamate transporter 1; NF200, neurofilament heavy polypeptide (200 kDa).