Specific disruption of a schwann cell dystrophin-related protein complex in a demyelinating neuropathy (Q28587855)

31 January 2017

title

Specific disruption of a schwann cell dystrophin-related protein complex in a demyelinating neuropathy (English)

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Dystrophin related protein 2

31 January 2017

main subject

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GOA release 2020-03-11

Dystroglycan 1

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GOA release 2020-03-11

Periaxin

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GOA release 2020-03-11

Schwann cell

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author name string

D. L. Sherman

1

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31 January 2017

C. Fabrizi

2

1 reference

31 January 2017

C. S. Gillespie

3

1 reference

31 January 2017

P. J. Brophy

4

1 reference

31 January 2017

language of work or name

English

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publication date

1 June 2001

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31 January 2017

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31 January 2017

volume

30

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31 January 2017

issue

3

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31 January 2017

page(s)

677–687

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The three human syntrophin genes are expressed in diverse tissues, have distinct chromosomal locations, and each bind to dystrophin and its relatives

31 January 2017

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Periaxin mutations cause recessive Dejerine-Sottas neuropathy

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Schwann cell function depends upon axonal signals and basal lamina components

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Evidence that contact with connective tissue matrix is required for normal interaction between Schwann cells and nerve fibers

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Linkage between axonal ensheathment and basal lamina production by Schwann cells

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Requirement for the PDZ domain protein, INAD, for localization of the TRP store-operated channel to a signaling complex

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A G+1-->A transversion at the 5' splice site of intron 69 of the dystrophin gene causing the absence of peripheral nerve Dp116 and severe clinical involvement in a DMD patient.

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PDZ motifs in PTP-BL and RIL bind to internal protein segments in the LIM domain protein RIL.

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Expression of the dystrophin-related protein 2 (Drp2) transcript in the mouse

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Non-muscle alpha-dystroglycan is involved in epithelial development

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Two PDZ domain proteins encoded by the murine periaxin gene are the result of alternative intron retention and are differentially targeted in Schwann cells

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Axonal regulation of Schwann cell integrin expression suggests a role for alpha 6 beta 4 in myelination.

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Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle

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PDZ domains in synapse assembly and signalling

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7 January 2021

Periaxin, a novel protein of myelinating Schwann cells with a possible role in axonal ensheathment

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Peripheral demyelination and neuropathic pain behavior in periaxin-deficient mice

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Reduced levels of dystrophin associated proteins in the brains of mice deficient for Dp71.

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A mutation in periaxin is responsible for CMT4F, an autosomal recessive form of Charcot-Marie-Tooth disease

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Unexpected modes of PDZ domain scaffolding revealed by structure of nNOS-syntrophin complex

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A sarcoglycan-dystroglycan complex anchors Dp116 and utrophin in the peripheral nervous system

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Identification and characterization of the dystrophin anchoring site on beta-dystroglycan

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A PDZ-containing scaffold related to the dystrophin complex at the basolateral membrane of epithelial cells

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Interaction of ion channels and receptors with PDZ domain proteins

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TRP and the PDZ protein, INAD, form the core complex required for retention of the signalplex in Drosophila photoreceptor cells

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Differential expression of dystrophin, utrophin and dystrophin-associated proteins in peripheral nerve

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An epitope structure for the C-terminal domain of dystrophin and utrophin

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Dystrophin protects the sarcolemma from stresses developed during muscle contraction

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Characterization of the transmembrane molecular architecture of the dystroglycan complex in schwann cells

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Dystrophin is not a specific component of the cardiac costamere

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Identifiers

DOI

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1 reference