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Article

Structural determinants of interaction, trafficking and function in the ClC-2/MLC1 subunit GlialCAM involved in leukodystrophy.

The Journal of physiology - 15 Sept 2015

Capdevila-Nortes Xavier, Jeworutzki Elena, Elorza-Vidal Xabier, Barrallo-Gimeno Alejandro, Pusch Michael, Estévez Raúl

Abstract excerpt

KEY POINTS: The extracellular domain of GlialCAM is necessary for its targeting to cell junctions, as well as for interactions with itself and MLC1 and ClC-2. The C-terminus of GlialCAM is not necessary for interaction but is required for targeting to cell junctions. The first three residues of the transmembrane segment of GlialCAM are required for GlialCAM-mediated ClC-2 activation. ABSTRACT: Mutations in the...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 26033718
DOI: 10.1113/JP270467
PMCID: PMC4594291

Genes

MLC1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

Related research

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Structural determinants of interaction, trafficking and function in the ClC-2/MLC1 subunit GlialCAM involved in leukodystrophy.PMID 26033718Read ↗

GlialCAM, a protein defective in a leukodystrophy, serves as a ClC-2 Cl(-) channel auxiliary subunit.Semantic proximity Read ↗

Leukoencephalopathy-causing CLCN2 mutations are associated with impaired Cl- channel function and trafficking.Semantic proximity Read ↗

Insights into MLC pathogenesis: GlialCAM is an MLC1 chaperone required for proper activation of volume-regulated anion currents.Semantic proximity Read ↗

Identification of the GlialCAM interactome: the G protein-coupled receptors GPRC5B and GPR37L1 modulate megalencephalic leukoencephalopathy proteins.Semantic proximity Read ↗

GlialCAM, a CLC-2 Cl(-) channel subunit, activates the slow gate of CLC chloride channels.Semantic proximity Read ↗

Comparison of zebrafish and mice knockouts for Megalencephalic Leukoencephalopathy proteins indicates that GlialCAM/MLC1 forms a functional unit.Semantic proximity Read ↗

Regulation of the orphan G-protein-coupled receptor GPRC5B by MLC1 and the cell adhesion molecule GlialCAM in megalencephalic leukoencephalopathy.Semantic proximity Read ↗

Structural basis for the dominant or recessive character of GLIALCAM mutations found in leukodystrophies.Semantic proximity Read ↗

Megalencephalic leukoencephalopathy with subcortical cysts protein 1 regulates glial surface localization of GLIALCAM from fish to humans.Semantic proximity Read ↗

Molecular mechanisms of MLC1 and GLIALCAM mutations in megalencephalic leukoencephalopathy with subcortical cysts.Semantic proximity Read ↗

Expanding the spectrum of megalencephalic leukoencephalopathy with subcortical cysts in two patients with GLIALCAM mutations.Semantic proximity Read ↗

MLC1: A New Calcium-regulated Protein Conferring Calcium Dependence to Volume-regulated Anion Channels (VRAC) in Astrocytes.Semantic proximity Read ↗

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