GlialCAM, a protein defective in a leukodystrophy, serves as a ClC-2 Cl(-) channel auxiliary subunit.
about
Discovery of CLC transport proteins: cloning, structure, function and pathophysiologyMegalencephalic leukoencephalopathy with subcortical cysts protein-1 modulates endosomal pH and protein trafficking in astrocytes: relevance to MLC disease pathogenesisBasolateral sorting of chloride channel 2 is mediated by interactions between a dileucine motif and the clathrin adaptor AP-1.Autism-epilepsy phenotype with macrocephaly suggests PTEN, but not GLIALCAM, genetic screening.Research and progress on ClC‑2 (Review).GlialCAM, a CLC-2 Cl(-) channel subunit, activates the slow gate of CLC chloride channels.CLC channel function and dysfunction in health and disease.CLC anion channel regulatory phosphorylation and conserved signal transduction domainsBrain connexins in demyelinating diseases: therapeutic potential of glial targetsA designed inhibitor of a CLC antiporter blocks function through a unique binding mode.Regulatory phosphorylation induces extracellular conformational changes in a CLC anion channelHepaCAM associates with connexin 43 and enhances its localization in cellular junctions.Severe changes in colon epithelium in the Mecp2-null mouse model of Rett syndrome.Chloride channelopathies of ClC-2.ASTROCYTES: EMERGING STARS IN LEUKODYSTROPHY PATHOGENESIS.Mutational consequences of aberrant ion channels in neurological disorders.Regulatory-auxiliary subunits of CLC chloride channel-transport proteins.MLC1 protein: a likely link between leukodystrophies and brain channelopathies.Structural determinants of interaction, trafficking and function in the ClC-2/MLC1 subunit GlialCAM involved in leukodystrophy.Megalencephalic leukoencephalopathy with subcortical cysts protein 1 regulates glial surface localization of GLIALCAM from fish to humans.ClC Channels and Transporters: Structure, Physiological Functions, and Implications in Human Chloride Channelopathies.Leukodystrophies: a proposed classification system based on pathological changes and pathogenetic mechanisms.Megalencephalic leukoencephalopathy with cysts: the Glialcam-null mouse modelRegulation of ClC-2 gating by intracellular ATP.Common gating of both CLC transporter subunits underlies voltage-dependent activation of the 2Cl-/1H+ exchanger ClC-7/Ostm1.Role of CBS and Bateman Domains in Phosphorylation-Dependent Regulation of a CLC Anion Channel.Regulatory Conformational Coupling between CLC Anion Channel Membrane and Cytoplasmic Domains.Photostimulation of whole-cell conductance in primary rat neocortical astrocytes mediated by organic semiconducting thin films.Expanding the spectrum of megalencephalic leukoencephalopathy with subcortical cysts in two patients with GLIALCAM mutations.Insights into MLC pathogenesis: GlialCAM is an MLC1 chaperone required for proper activation of volume-regulated anion currents.Identification and characterization of the zebrafish ClC-2 chloride channel orthologs.Oxygen and Glucose Deprivation Induces Bergmann Glia Membrane Depolarization and Ca2+ Rises Mainly Mediated by K+ and ATP Increases in the Extracellular Space.Identification and characterization of the three members of the CLC family of anion transport proteins in Trypanosoma brucei.Leukoencephalopathy-causing CLCN2 mutations are associated with impaired Cl- channel function and trafficking.The signaling role for chloride in the bidirectional communication between neurons and astrocytes.CLCN2 chloride channel mutations in familial hyperaldosteronism type II.Mice with megalencephalic leukoencephalopathy with cysts: a developmental angle.Astrocyte-mediated infantile-onset leukoencephalopathy mouse model.Identification and Functional Characterization of CLCN1 Mutations Found in Nondystrophic Myotonia Patients.Functional analyses of mutations in HEPACAM causing megalencephalic leukoencephalopathy.
P2860
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P2860
GlialCAM, a protein defective in a leukodystrophy, serves as a ClC-2 Cl(-) channel auxiliary subunit.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
GlialCAM, a protein defective ...... (-) channel auxiliary subunit.
@ast
GlialCAM, a protein defective ...... (-) channel auxiliary subunit.
@en
type
label
GlialCAM, a protein defective ...... (-) channel auxiliary subunit.
@ast
GlialCAM, a protein defective ...... (-) channel auxiliary subunit.
@en
prefLabel
GlialCAM, a protein defective ...... (-) channel auxiliary subunit.
@ast
GlialCAM, a protein defective ...... (-) channel auxiliary subunit.
@en
P2093
P2860
P50
P1433
P1476
GlialCAM, a protein defective ...... (-) channel auxiliary subunit.
@en
P2093
Catrin S Müller
Elena Jeworutzki
Giovanni Zifarelli
Raúl Estévez
Sònia Sirisi
Tania López-Hernández
Tanit Arnedo
Uwe Schulte
Virginia Nunes
Xavier Capdevila-Nortes
P2860
P304
P356
10.1016/J.NEURON.2011.12.039
P407
P577
2012-03-01T00:00:00Z