Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice. - Wikidata - awgldk - TriplyDB

Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice.

Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice.

http://www.wikidata.org/entity/Q43076742

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Functional Role of the Disulfide Isomerase ERp57 in Axonal Regeneration Divergent effects of PERK and IRE1 signaling on cell viability Seipinopathy: a novel endoplasmic reticulum stress-associated disease Two novel missense mutations in the myelin protein zero gene causes Charcot-Marie-Tooth type 2 and Déjérine-Sottas syndrome.P0 (protein zero) mutation S34C underlies instability of internodal myelin in S63C mice.Regulation of basal cellular physiology by the homeostatic unfolded protein response The deadly connection between endoplasmic reticulum, Ca2+, protein synthesis, and the endoplasmic reticulum stress response in malignant glioma cells.Macroautophagy is regulated by the UPR-mediator CHOP and accentuates the phenotype of SBMA mice.The molecular machinery of myelin gene transcription in Schwann cells.Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit A genetic screen identifies Tor as an interactor of VAPB in a Drosophila model of amyotrophic lateral sclerosis.Insulinlike growth factor (IGF)-1 administration ameliorates disease manifestations in a mouse model of spinal and bulbar muscular atrophy Using temporal genetic switches to synchronize the unfolded protein response in cell populations in vivo.Loss of Fig4 in both Schwann cells and motor neurons contributes to CMT4J neuropathy.P0S63del impedes the arrival of wild-type P0 glycoprotein to myelin in CMT1B mice.Endoplasmic reticulum stress in disorders of myelinating cells.The unfolded protein response is a major mechanism by which LRP1 regulates Schwann cell survival after injury.Unfolding the unfolded protein response: unique insights into brain ischemia.Colony-stimulating factor-1 mediates macrophage-related neural damage in a model for Charcot-Marie-Tooth disease type 1X Attenuating the endoplasmic reticulum stress response improves functional recovery after spinal cord injury.Genotype-phenotype characteristics and baseline natural history of heritable neuropathies caused by mutations in the MPZ gene.Induction of endoplasmic reticulum stress genes, BiP and chop, in genetic and environmental models of retinal degeneration Induction of heat shock protein 70 (Hsp70) prevents neuregulin-induced demyelination by enhancing the proteasomal clearance of c-Jun.Mutation of sec63 in zebrafish causes defects in myelinated axons and liver pathology Molecular genetics of charcot-marie-tooth disease: from genes to genomes.Loss of HtrA2/Omi activity in non-neuronal tissues of adult mice causes premature aging.Activation of the unfolded protein response promotes axonal regeneration after peripheral nerve injury Aberrant Schwann cell lipid metabolism linked to mitochondrial deficits leads to axon degeneration and neuropathy.Resetting translational homeostasis restores myelination in Charcot-Marie-Tooth disease type 1B mice A modified UPR stress sensing system reveals a novel tissue distribution of IRE1/XBP1 activity during normal Drosophila development.Inherited peripheral neuropathies.Perk Ablation Ameliorates Myelination in S63del-Charcot-Marie-Tooth 1B Neuropathy.Rescue of Glaucomatous Neurodegeneration by Differentially Modulating Neuronal Endoplasmic Reticulum Stress Molecules.Update on Charcot-Marie-Tooth disease.Molecular mechanisms of inherited demyelinating neuropathies.Enhanced integrated stress response promotes myelinating oligodendrocyte survival in response to interferon-gamma ER-stress-induced transcriptional regulation increases protein synthesis leading to cell death.Overexpression of CHOP in Myelinating Cells Does Not Confer a Significant Phenotype under Normal or Metabolic Stress Conditions.Phenotypic presentation of the Ser63Del MPZ mutation The degree of folding instability of the envelope protein of a neurovirulent murine retrovirus correlates with the severity of the neurological disease.

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Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot-Marie-Tooth 1B mice.

http://www.wikidata.org/entity/Q43076742

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Ablation of the UPR-mediator C ...... n Charcot-Marie-Tooth 1B mice.

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Ablation of the UPR-mediator C ...... n Charcot-Marie-Tooth 1B mice.

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Ablation of the UPR-mediator C ...... n Charcot-Marie-Tooth 1B mice.

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Ablation of the UPR-mediator C ...... n Charcot-Marie-Tooth 1B mice.

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J.NEURON.2007.12.021

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Elisa Tinelli

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P2860

Caspase-12 mediates endoplasmic-reticulum-specific apoptosis and cytotoxicity by amyloid-beta

Endoplasmic reticulum oxidoreductin 1-lbeta (ERO1-Lbeta), a human gene induced in the course of the unfolded protein response

XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor

ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats

Deletion of the serine 34 codon from the major peripheral myelin protein P0 gene in Charcot-Marie-Tooth disease type 1B

Mutations linked to leukoencephalopathy with vanishing white matter impair the function of the eukaryotic initiation factor 2B complex in diverse ways

Identification of novel stress-induced genes downstream of chop

Mutations causing childhood ataxia with central nervous system hypomyelination reduce eukaryotic initiation factor 2B complex formation and activity

Crystal structure of the extracellular domain from P0, the major structural protein of peripheral nerve myelin

Signal integration in the endoplasmic reticulum unfolded protein response

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