Genetic Background Influences UPR but not PLP Processing in the rumpshaker Model of PMD/SPG2
M McLaughlin, SA Karim, P Montague, JA Barrie… - Neurochemical …, 2007 - Springer
… 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 …
little difference in PLP processing between the two phenotypes. Processing of rumpshaker PLP …
Processing of PLP in a model of Pelizaeus‐Merzbacher disease/SPG2 due to the rumpshaker mutation
M McLaughlin, JA Barrie, S Karim, P Montague… - Glia, 2006 - Wiley Online Library
… 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 …
suggesting the influence of other … Using a model of the mild form of PMD/SPG2, we show that …
Transgenic complementation of rumpshaker with wild type Proteolipid protein
JA Barrie - 2008 - theses.gla.ac.uk
… 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 …
occurs at varying times in the … The study examines the effect of normalised PLP on the UPR …
[HTML][HTML] Cellular pathology of Pelizaeus-Merzbacher disease involving chaperones associated with endoplasmic reticulum stress
K Inoue - Frontiers in molecular biosciences, 2017 - frontiersin.org
… 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, …
with an I187T mutation serve as … the PLP1 overexpression does not involve ER stress or UPR, …
[HTML][HTML] Endoplasmic reticulum protein quality control failure in myelin disorders
VG Volpi, T Touvier, M D'Antonio - Frontiers in molecular …, 2017 - frontiersin.org
… 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 …
then that the genetic ablation … (but not the PMP22 Tr protein), activate the UPR response in vitro …
The molecular and cellular defects underlying Pelizaeus–Merzbacher disease
KJ Woodward - Expert reviews in molecular medicine, 2008 - cambridge.org
… 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…
background influences UPR but not PLP1 processing in the rumpshaker model of PMD/SPG2…
Pelizaeus-Merzbacher disease: genetic and cellular pathogenesis
JY Garbern - Cellular and Molecular Life Sciences, 2007 - Springer
… 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…
mechanisms that cause PMD/SPG2 is … determines phenotypic severity of the Plp rumpshaker muta…
Endoplasmic reticulum stress in disorders of myelinating cells
… 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 …
PLP proteins are not properly … shows that the UPR is involved in the demyelination process …
[HTML][HTML] Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice
… 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 …
UPR … Altogether, these data show that BiP, CHOP, and its target genes are upregulated in …
Overview of Genetic Causes of Spasticity in Adults and Children
… 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. …
background influences UPR but not PLP processing in the rumpshaker model of PMD/SPG2. …