… that facilitate this process are still being elucidated. … DM20 is a gene splice variant of PLP
expressed on the surface of … time-point and UPR target each significantly influenced this (Fig. 7b…

… between the different genetic backgrounds was not possible, … CHOP null background, but
during this process we enriched … known to customise their UPR during differentiation and in …

… lipids and incorporation of PLP/DM20 are all also affected in … #66 mice and patients with
PMD does not appear to be due to one … of such hormones in females may have an influence on …

… Panels show normal myelinating processes at 0, 3, 6, 12 and … mutations in human PMD/SPG2
patients with corresponding … , PLP1 overexpression does not activate the UPR, but leads to …

… /Plp1 gene, has been proposed to have functions that are … induce an unfolded protein response
(UPR). In contrast to … Our data shows that increased production of PLP has wide ranging …

… It is suggested that genetic background may have a strong influence in the … Genetic
background influences UPR but not PLP processing in the rumpshaker model of PMD/SPG2. …

… the cartilage growth plate influences the UPR resulting from … It is therefore not surprising
that the XBP1 branch of the UPR … ”) and in the UPR (“protein processing in the endoplasmic …

… time, that mice with duplications of the Plp1 gene exhibit major … PLP is in vesicles that is
transported into processes. Scale … deficient and rumpshaker, but markers of an UPR are, at best, …

… apoptosis via unfolded-protein response (UPR), which in turn may … Naturally occurring
mouse mutants such as rumpshaker … with some of the point mutations which lead to PMD/SPG2. …

… these processes, the UPR … gene induce ER stress differently and affect clinical severity.
Factors, such as retention of misfolded proteins or the extent of UPR activation, may influence …