Desert hedgehog is a mediator of demyelination in compression neuropathies.

摘要: The secreted protein desert hedgehog (dhh) controls the formation of nerve perineurium during development and is a key component Schwann cells that ensures peripheral survival. We postulated dhh may play critical role in maintaining myelination investigated its demyelination-induced compression neuropathies by using post-natal model chronic injury wildtype dhh(-/-) mice. evaluated demyelination electrophysiological, morphological, molecular approaches. transcripts are down-regulated early after wild-type mice, suggesting an intimate relationship between pathway demyelination. In induced more prominent severe relative to their counterparts, protective dhh. Alterations fiber characteristics included significant decreases conduction velocity, increased myelin debris, substantial internodal length. Furthermore, vitro studies showed blockade via either adenovirus-mediated (shRNA) or pharmacological inhibition both resulted demyelination, which could be rescued exogenous Dhh. Exogenous Dhh was against this maintained at baseline levels custom bioreactor applied biophysical forces myelinated DRG/Schwann cell co-cultures. Together, these results demonstrate pivotal for myelination. signaling reveals potential target therapeutic intervention prevent treat nerves neuropathies.