(Q82679783)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:16506223%20AND%20SRC:MED&resulttype=core&format=json

17 January 2020

title

Processing of PLP in a model of Pelizaeus-Merzbacher disease/SPG2 due to the rumpshaker mutation (English)

1 reference

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17 January 2020

5

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17 January 2020

author name string

Mark McLaughlin

1

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17 January 2020

Jennifer A Barrie

2

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17 January 2020

Saadia Karim

3

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17 January 2020

Paul Montague

4

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17 January 2020

Douglas Kirkham

6

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17 January 2020

Christine E Thomson

7

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17 January 2020

Ian R Griffiths

8

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17 January 2020

publication date

1 May 2006

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17 January 2020

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17 January 2020

volume

53

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17 January 2020

issue

7

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17 January 2020

page(s)

715-722

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Genetic background determines phenotypic severity of the Plp rumpshaker mutation.

17 January 2020

cites work

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Mdm-2 and ubiquitin-independent p53 proteasomal degradation regulated by NQO1.

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Misfolded BiP is degraded by a proteasome-independent endoplasmic-reticulum-associated degradation pathway

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Abnormal compact myelin in the myelin-deficient rat: absence of proteolipid protein correlates with a defect in the intraperiod line

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Survival of, and competition between, oligodendrocytes expressing different alleles of the Plp gene.

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Quality control in the endoplasmic reticulum

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Patients lacking the major CNS myelin protein, proteolipid protein 1, develop length-dependent axonal degeneration in the absence of demyelination and inflammation.

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Protein translation and folding are coupled by an endoplasmic-reticulum-resident kinase

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Pelizaeus-Merzbacher disease and spastic paraplegia type 2: two faces of myelin loss from mutations in the same gene

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Dominant negative pathogenesis by mutant proinsulin in the Akita diabetic mouse

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Endoplasmic reticulum-associated protein degradation

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Monoclonal antibody O10 defines a conformationally sensitive cell-surface epitope of proteolipid protein (PLP): evidence that PLP misfolding underlies dysmyelination in mutant mice.

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Deubiquitinating enzymes as cellular regulators

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Aspartoacylase is restricted primarily to myelin synthesizing cells in the CNS: therapeutic implications for Canavan disease

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Assembly of CNS myelin in the absence of proteolipid protein

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

The rumpshaker mutation in spastic paraplegia

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Selective inhibitors of the proteasome-dependent and vacuolar pathways of protein degradation in Saccharomyces cerevisiae

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Evidence for possible interactions between PLP and DM20 within the myelin sheath

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Cytoskeletal and nuclear localization of myelin oligodendrocytic basic protein isoforms

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

A male child with the rumpshaker mutation, X-linked spastic paraplegia/Pelizaeus-Merzbacher disease and lysinuria.

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Lactacystin, a specific inhibitor of the proteasome, induces apoptosis and activates caspase-3 in cultured cerebellar granule cells

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

A trip to the ER: coping with stress

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Endoplasmic reticulum-associated degradation: exceptions to the rule

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Morphometric analysis of normal, mutant, and transgenic CNS: correlation of myelin basic protein expression to myelinogenesis

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Ubiquitin conjugation is not required for the degradation of oxidized proteins by proteasome.

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Programmed cell death in the dysmyelinating mutants

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

The early phenotype associated with the jimpy mutation of the proteolipid protein gene

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Observations on the structure of myelin lacking the major proteolipid protein

1 reference

https://api.crossref.org/works/10.1002%2FGLIA.20325

7 January 2021

Identifiers

DOI

10.1002/GLIA.20325

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:16506223%20AND%20SRC:MED&resulttype=core&format=json

17 January 2020

1 reference