The unfolded protein response modulates disease severity in Pelizaeus-Merzbacher disease. (Q36155932)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12441049%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

title

The unfolded protein response modulates disease severity in Pelizaeus-Merzbacher disease (English)

1 reference

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15 December 2019

4

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15 December 2019

author name string

Cherie M Southwood

series ordinal

1

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15 December 2019

James Garbern

series ordinal

2

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15 December 2019

Wei Jiang

series ordinal

3

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15 December 2019

language of work or name

English

0 references

publication date

1 November 2002

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15 December 2019

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15 December 2019

volume

36

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15 December 2019

page(s)

585-596

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15 December 2019

issue

4

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Distinct roles of activating transcription factor 6 (ATF6) and double-stranded RNA-activated protein kinase-like endoplasmic reticulum kinase (PERK) in transcription during the mammalian unfolded protein response

15 December 2019

cites work

1 reference

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Overexpression of the myelin proteolipid protein leads to accumulation of cholesterol and proteolipid protein in endosomes/lysosomes: implications for Pelizaeus-Merzbacher disease

2 October 2017

1 reference

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Targeted disruption of the Chop gene delays endoplasmic reticulum stress-mediated diabetes

2 October 2017

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The unfolding tale of the unfolded protein response

2 October 2017

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Diabetes mellitus and exocrine pancreatic dysfunction in perk-/- mice reveals a role for translational control in secretory cell survival

2 October 2017

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Molecular pathways of oligodendrocyte apoptosis revealed by mutations in the proteolipid protein gene

2 October 2017

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Upregulation of BiP and CHOP by the unfolded-protein response is independent of presenilin expression.

2 October 2017

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Regulated translation initiation controls stress-induced gene expression in mammalian cells

2 October 2017

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Functional and genomic analyses reveal an essential coordination between the unfolded protein response and ER-associated degradation

2 October 2017

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CNS myelin and sertoli cell tight junction strands are absent in Osp/claudin-11 null mice

2 October 2017

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Presenilin-1 mutations downregulate the signalling pathway of the unfolded-protein response

2 October 2017

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The molecular pathogenesis of Pelizaeus-Merzbacher disease

2 October 2017

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Integration of endoplasmic reticulum signaling in health and disease

2 October 2017

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Identification of novel stress-induced genes downstream of chop

2 October 2017

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CHOP is implicated in programmed cell death in response to impaired function of the endoplasmic reticulum

2 October 2017

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Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease.

2 October 2017

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In vitro death of jimpy oligodendrocytes: correlation with onset of DM-20/PLP expression and resistance to oligodendrogliotrophic factors

2 October 2017

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A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease.

2 October 2017

1 reference

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Transgenic systems in studying myelin gene expression

2 October 2017

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Dominant-negative action of the jimpy mutation in mice complemented with an autosomal transgene for myelin proteolipid protein

2 October 2017

1 reference

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Aberrant splicing of proteolipid protein mRNA in the dysmyelinating jimpy mutant mouse

2 October 2017

1 reference

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Human 3 beta-hydroxysteroid dehydrogenase/delta 5----4isomerase from placenta: expression in nonsteroidogenic cells of a protein that catalyzes the dehydrogenation/isomerization of C21 and C19 steroids

2 October 2017

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Rumpshaker mouse: a new X-linked mutation affecting myelination: evidence for a defect in PLP expression

2 October 2017

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Isolation of biologically active ribonucleic acid from sources enriched in ribonuclease

2 October 2017

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A role for presenilin-1 in nuclear accumulation of Ire1 fragments and induction of the mammalian unfolded protein response

2 October 2017

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Major histocompatibility complex class I expression in oligodendrocytes induces hypomyelination in transgenic mice.

2 July 2018

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Dysmyelination in transgenic mice resulting from expression of class I histocompatibility molecules in oligodendrocytes.

2 July 2018

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Monoclonal antibodies against myelin proteolipid protein: identification and characterization of two major determinants.

2 July 2018

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Uncoupling of hypomyelination and glial cell death by a mutation in the proteolipid protein gene

2 July 2018

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Rumpshaker: an X-linked mutation causing hypomyelination: developmental differences in myelination and glial cells between the optic nerve and spinal cord.

2 July 2018

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The evolution of lipophilin genes from invertebrates to tetrapods: DM-20 cannot replace proteolipid protein in CNS myelin.

2 July 2018

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Axonal swellings and degeneration in mice lacking the major proteolipid of myelin.

23 September 2018

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Many naturally occurring mutations of myelin proteolipid protein impair its intracellular transport.

23 September 2018

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Intracellular transport and sorting of the oligodendrocyte transmembrane proteolipid protein.

23 September 2018

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Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4603660

Conservative amino acid substitution in the myelin proteolipid protein of jimpymsd mice

23 September 2018

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12 December 2020

Jimpy(msd) mouse mutation and connatal Pelizaeus-Merzbacher disease

1 reference

12 December 2020

The rumpshaker mutation in spastic paraplegia

1 reference

12 December 2020

Major histocompatibility complex heavy chain accumulation in the endoplasmic reticulum of oligodendrocytes results in myelin abnormalities

1 reference

12 December 2020

Glial cell degeneration and hypomyelination caused by overexpression of myelin proteolipid protein gene

1 reference

12 December 2020

Two distinct stress signaling pathways converge upon the CHOP promoter during the mammalian unfolded protein response

1 reference

12 December 2020

Neonatal hepatitis induced by alpha 1-antitrypsin: a transgenic mouse model

1 reference

12 December 2020

Dissection of the phenotype and genotype of the X-linked myelin mutants

1 reference

12 December 2020

The COS-7 cell in vitro paradigm to study myelin proteolipid protein 1 gene mutations

1 reference

12 December 2020

Mutations in noncoding regions of the proteolipid protein gene in Pelizaeus-Merzbacher disease

1 reference

12 December 2020

Oligodendroglial cell death in jimpy mice: an explanation for the myelin deficit

1 reference

12 December 2020

Myelin structure in proteolipid protein (PLP)-null mouse spinal cord

1 reference

12 December 2020

Conservation of topology, but not conformation, of the proteolipid proteins of the myelin sheath

1 reference

12 December 2020

Assembly of CNS myelin in the absence of proteolipid protein

1 reference

12 December 2020

Diabetes in transgenic mice resulting from over-expression of class I histocompatibility molecules in pancreatic β cells

1 reference

12 December 2020

Major histocompatibility complex Class I expression in oligodendrocytes induces hypomyelination in transgenic mice

1 reference

Crossref

https://api.crossref.org/works/10.1016%2FS0896-6273%2802%2901045-0

7 January 2021

inferred from DOI database lookup

Identifiers

DOI

10.1016/S0896-6273(02)01045-0

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12441049%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:12441049%20AND%20SRC:MED&resulttype=core&format=json

15 December 2019

1 reference