Phenotypic severity of murine Plp mutants reflects in vivo and in vitro variations in transport of PLP isoproteins.

Phenotypic severity of murine Plp mutants reflects in vivo and in vitro variations in transport of PLP isoproteins.

Item
http://www.wikidata.org/entity/Q41097292
Different proteolipid protein mutants exhibit unique metabolic defects.Involvement of ER stress in dysmyelination of Pelizaeus-Merzbacher Disease with PLP1 missense mutations shown by iPSC-derived oligodendrocytes.Membrane traffic in myelinating oligodendrocytes.CNS myelination and PLP gene dosage.Insertion of mutant proteolipid protein results in missorting of myelin proteins.Novel alternatively spliced endoplasmic reticulum retention signal in the cytoplasmic loop of Proteolipid Protein-1.Differences in endoplasmic-reticulum quality control determine the cellular response to disease-associated mutants of proteolipid protein.Trafficking of PLP/DM20 and cAMP signaling in immortalized jimpy oligodendrocytes.Differential sensitivity in the survival of oligodendrocyte cell lines to overexpression of myelin proteolipid protein gene products.An immortalized jimpy oligodendrocyte cell line: defects in cell cycle and cAMP pathway.Cellular mechanisms of connexin32 mutations associated with CNS manifestations.Evidence for possible interactions between PLP and DM20 within the myelin sheath.Current concepts of PLP and its role in the nervous system.Mouse models of myelin diseases.Effects of the jimpy mutation on mouse retinal structure and function.Different mutations in the same codon of the proteolipid protein gene, PLP, may help in correlating genotype with phenotype in Pelizaeus-Merzbacher disease/X-linked spastic paraplegia (PMD/SPG2).
P2860

Phenotypic severity of murine Plp mutants reflects in vivo and in vitro variations in transport of PLP isoproteins.

Item
http://www.wikidata.org/entity/Q41097292
description
1997 nî lūn-bûn
@nan
1997年の論文
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1997年論文
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1997年論文
@zh-hant
1997年論文
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1997年論文
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1997年論文
@zh-tw
1997年论文
@wuu
1997年论文
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1997年论文
@zh-cn
name
Phenotypic severity of murine ...... transport of PLP isoproteins.
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type
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Phenotypic severity of murine ...... transport of PLP isoproteins.
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Phenotypic severity of murine ...... transport of PLP isoproteins.
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Phenotypic severity of murine ...... transport of PLP isoproteins.
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10.1002/(SICI)1098-1136(199708)20:4<322::AID-GLIA5>3.0.CO;2-7
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