@article{discovery10098423,
            note = {This article is available under a Creative Commons License (Attribution 4.0 International, as described at https://creativecommons.org/licenses/by/4.0/).},
           title = {Neuronal activity disrupts myelinated axon integrity in the absence of NKCC1b},
            year = {2020},
          volume = {219},
           month = {July},
          number = {7},
         journal = {Journal of Cell Biology},
        abstract = {Through a genetic screen in zebrafish, we identified a mutant with disruption to myelin in both the CNS and PNS caused by a mutation in a previously uncharacterized gene, slc12a2b, predicted to encode a Na+, K+, and Cl? (NKCC) cotransporter, NKCC1b. slc12a2b/NKCC1b mutants exhibited a severe and progressive pathology in the PNS, characterized by dysmyelination and swelling of the periaxonal space at the axon-myelin interface. Cell-type-specific loss of slc12a2b/NKCC1b in either neurons or myelinating Schwann cells recapitulated these pathologies. Given that NKCC1 is critical for ion homeostasis, we asked whether the disruption to myelinated axons in slc12a2b/NKCC1b mutants is affected by neuronal activity. Strikingly, we found that blocking neuronal activity completely prevented and could even rescue the pathology in slc12a2b/NKCC1b mutants. Together, our data indicate that NKCC1b is required to maintain neuronal activity-related solute homeostasis at the axon-myelin interface, and the integrity of myelinated axons.},
             url = {https://doi.org/10.1083/jcb.201909022},
          author = {Marshall-Phelps, KLH and Kegel, L and Baraban, M and Ruhwedel, T and Almeida, RG and Rubio-Brotons, M and Klingseisen, A and Benito-Kwiecinski, SK and Early, JJ and Bin, JM and Suminaite, D and Livesey, MR and M{\"o}bius, W and Poole, RJ and Lyons, DA},
        keywords = {Cell signaling}
}