… in processing of PLP/DM20 or differences in the UPR are associated with … that there is
little difference in PLP processing between the two phenotypes. Processing of rumpshaker PLP …

… MBP, although the mechanisms are not yet defined but are likely to … on genetic background,
suggesting the influence of other … Using a model of the mild form of PMD/SPG2, we show that …

… It is only recently that their influence on the functions of the … Myelination is a process that
occurs at varying times in the … The study examines the effect of normalised PLP on the UPR …

… to the production of aberrant polypeptides that accumulate in … The rumpshaker (rsh) mice
with an I187T mutation serve as … the PLP1 overexpression does not involve ER stress or UPR, …

… cancer, but also to those physiological processes in which … It should come as no surprise
then that the genetic ablation … (but not the PMP22 Tr protein), activate the UPR response in vitro …

… PMD/SPG2 phenotypes range from severe connatal PMD through an intermediate … Genetic
background influences UPR but not PLP1 processing in the rumpshaker model of PMD/SPG2…

… noted that rsh can be influenced by genetic background effects… genetic and cellular
mechanisms that cause PMD/SPG2 is … determines phenotypic severity of the Plp rumpshaker muta…

… PNS that are caused by mutations in a number of distinct genes … have shown that the mutant
PLP proteins are not properly … shows that the UPR is involved in the demyelination process …

… that Schwann cell death follows, not precedes demyelination, we propose that the maladaptive
UPR … Altogether, these data show that BiP, CHOP, and its target genes are upregulated in …

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