ArrayExpress Uploaderarrayexpress_sid8645Postnatal day 5WTPostnatal day 5WTPostnatal day 5CHOP nullPostnatal day 5CHOP nullPostnatal day 5P0S63delPostnatal day 5P0S63delPostnatal day 5P0S63del/CHOP nullPostnatal day 5P0S63del/CHOP nullPostnatal day 28WTPostnatal day 28WTPostnatal day 28CHOP nullPostnatal day 28CHOP nullPostnatal day 28P0S63delPostnatal day 28P0S63delPostnatal day 28P0S63del/CHOP nullPostnatal day 28P0S63del/CHOP null4 monthsWT4 monthsWT4 monthsCHOP null4 monthsCHOP null4 monthsP0S63del4 monthsP0S63del4 monthsP0S63del/CHOP null4 monthsP0S63del/CHOP null08We have generated mouse models of real CMT1B mutations in the gene encoding for myelin protein zero (P0). One of these mutants, P0S63del...23547100/profile/8773/arrayexpressuploader224diseasemyelinneuropathypointproteinDec.12, 2014Falseexpression-data-from-a-charcot-marie-tooth-1b-neurhttp://www.ebi.ac.uk/arrayexpress/experiments/E-GEOD-40610E-GEOD-40610_A-AFFY-36Expression data from a Charcot-Marie-Tooth 1B neuropathy mouse modelNov.24, 2014We have generated mouse models of real CMT1B mutations in the gene encoding for myelin protein zero (P0). One of these mutants, P0S63del is retained in the ER where it elicits an unfolded protein response (UPR). Genetic ablation of the UPR factor CHOP restores the motor capacity in S63del mice. We used microarray to decipher the molecular mechanism undelying the P0S63del neuropathy and the rescue in S63del/Chop null nerves. Sciatic nerves were dissected from WT, S63del, Chop null and S63del/Chop null mice at three different time points: (i) postnatal day 5 (P5) when myelination has just started and only the primary effects of the presence of the mutant protein should be detected; (ii) P28, around the peak of myelination, when all the downstream targets of CHOP should be activated; and (iii) 4 months, to check for secondary effects of the disease and because this was the time-point when the motor and morphological rescue due to the ablation of CHOP were clearly detectable.E-GEOD-40610mousehttp://www.ebi.ac.uk/arrayexpress/experiments/E-GEOD-40610/samples/