Indeed, the involvement of CD18/CD11a heterodimer in the adhesion of cytotoxic T cells to their target cells, including the delivery of a distinct signal essential for directing released granules to antigenbearing target cells to mediate their destruction, is unique. An alternative explanation for the dysfunctional cells is that expression of the other CD18/11 components werenot enough, thusa BAY 43-9006 higher level of readthrough would be required in order to restore appropriate localization of the CD18 protein and full functional activity. This speculation can provide a rationale for the development of future therapeutic modalities aiming to maximize the readthrough potential or to improve the function of the corrected protein at the cell surface. For example, Nudelman et al. recently described a novel NB54 aminoglycoside with reduced toxicity and enhanced suppression of disease-causing premature stop mutations in cells that originated from varioushereditarydiseases which improves both expression and function of the corrected protein. In summary, we presented a novel premature termination codon mutation in the CD18 gene of two Palestinian children with severe LAD1 phenotype. This mutation enabled us to test the proof of concept designed to readthrough premature stop mutations. We showed that a corrected full-length CD18 is produced as a result of gentamicin treatment both in vivo and in vitro, although the protein is either dysfunctional or mislocalized. In addition, we found that the integrity of the CD18/CD11 complex was impaired, possibly due to lack of CD11a expression. Our results should encourage the search for more effective aminoglycoside readthrough compounds to treat LAD1 and other potential genetic disorders caused by nonsense mutations. From birth onwards, the neonatal mammal must be able to breathe and adapt its breathing activity to environmental changes and behaviors. Therefore a correct function of the pontomedullary respiratory network is required at birth, not only for the elaboration of the respiratory rhythm but also for its adaptation to physiological needs. In neonates, the respiratory rhythm generator is composed of two coupled, interacting networks: the preBo¨tzinger complex which contains the primary rhythm generating neurons in brainstem slices and the parafacial respiratory group. The pFRG neurons express the transcription factor Phox2b, display a pre-inspiratory discharge and play a major role in detecting CO2/pH changes and adjusting the RRG activity in neonatal and juvenile animals. In embryos, organized rhythmic activities emerge in the preBo¨tC and pFRG as early as at embryonic day 15.5 and 14.5, respectively. After birth, the embryonic pFRG forms the retrotrapezoid nucleus with Phox2b glutamatergic neurons detecting changes.