Subcellular trafficking of proteolipid protein (PLP-DM20) and novel mechanisms of ER retention in Pelizaeus-Merzbacher disease
AS Dhaunchak - 2006 - pure.mpg.de
… treated a genuine animal model (rumpshaker mice) with Turmeric. … and information processing,
hence are required for normal … unfolded protein response (UPR) that includes ER growth …
hence are required for normal … unfolded protein response (UPR) that includes ER growth …
[HTML][HTML] Mesencephalic astrocyte-derived neurotropic factor is an important factor in chondrocyte ER homeostasis
… , thus indicating that MANF is not essential for the processing and … The influence of MANF
on tunicamycin-induced ER stress was … Here, we show that disrupting the chondrocyte UPR …
on tunicamycin-induced ER stress was … Here, we show that disrupting the chondrocyte UPR …
Loss of lysophosphatidic acid receptor LPA1 alters oligodendrocyte differentiation and myelination in the mouse cerebral cortex
B García-Díaz, R Riquelme, I Varela-Nieto… - Brain Structure and …, 2015 - Springer
… Because the PLP/DM20 antibody used in this study does not … subcellular deficits that
could affect the overall process of … genes are influenced by the state of myelination and gene-…
could affect the overall process of … genes are influenced by the state of myelination and gene-…
[图书][B] Modeling Genetic Diseases of Myelin Using Patient-derived Induced Pluripotent Stem Cells
ZS Nevin - 2017 - search.proquest.com
… As oligodendrocytes mature, they extend membrane processes that are … We hypothesize
that there is no all-inclusive cellular … only the rumpshaker mutation has also been identified in …
that there is no all-inclusive cellular … only the rumpshaker mutation has also been identified in …
[图书][B] Role of an intronic enhancer in the regulation of myelin proteolipid protein gene expression
F Meng - 2006 - search.proquest.com
… Causes of PMD/SPG2 include Pip point mutations, duplications … /DM20 mutant proteins
accumulate in the ER, provoke the UPR … The rumpshaker mutant is characterized by a missense …
accumulate in the ER, provoke the UPR … The rumpshaker mutant is characterized by a missense …
Quantitative measurement of events in the mammalian unfolded protein response
J Shang - Methods in enzymology, 2011 - Elsevier
… Thus, ATF6 and IRE1/XBP1 signaling may be necessary for gene activation, but they do
not define the magnitude of UPR-dependent mRNA increases, which appear dependent upon …
not define the magnitude of UPR-dependent mRNA increases, which appear dependent upon …
[图书][B] Novel synthesis and degradation pathways for the myelin proteolipid proteins
MA Cypher - 2003 - search.proquest.com
… Other rare PMD/SPG2 cases are associated with the … Only the PLP transgene was used
because the rumpshaker … PLP mutations have been shown to be strongly influenced by the …
because the rumpshaker … PLP mutations have been shown to be strongly influenced by the …
Pelizaeus-Merzbacher disease and spastic paraplegia type 2: two faces of myelin loss from mutations in the same gene
LD Hudson - Journal of child neurology, 2003 - journals.sagepub.com
… Pelizaeus-Merzbacher disease and X-linked spastic paraplegia type 2 are two sides of the
same coin. Both arise from mutations in the gene encoding myelin proteolipid protein. The …
same coin. Both arise from mutations in the gene encoding myelin proteolipid protein. The …