Defective expression of plectin/HD1 in epidermolysis bullosa simplex with muscular dystrophy
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Multiple functions of the integrin alpha6beta4 in epidermal homeostasis and tumorigenesisThe N terminus of the transmembrane protein BP180 interacts with the N-terminal domain of BP230, thereby mediating keratin cytoskeleton anchorage to the cell surface at the site of the hemidesmosomeNot just scaffolding: plectin regulates actin dynamics in cultured cellsBinding of integrin alpha6beta4 to plectin prevents plectin association with F-actin but does not interfere with intermediate filament bindingBasic amino acid residue cluster within nuclear targeting sequence motif is essential for cytoplasmic plectin-vimentin network junctionsLaminin 5 binds the NC-1 domain of type VII collagenPlectin sidearms mediate interaction of intermediate filaments with microtubules and other components of the cytoskeletonMicrotubule actin cross-linking factor (MACF): a hybrid of dystonin and dystrophin that can interact with the actin and microtubule cytoskeletonsHemidesmosome formation is initiated by the beta4 integrin subunit, requires complex formation of beta4 and HD1/plectin, and involves a direct interaction between beta4 and the bullous pemphigoid antigen 180The localization of bullous pemphigoid antigen 180 (BP180) in hemidesmosomes is mediated by its cytoplasmic domain and seems to be regulated by the beta4 integrin subunitDefining keratin protein function in skin epithelia: epidermolysis bullosa simplex and its aftermathTargeted proteolysis of plectin isoform 1a accounts for hemidesmosome dysfunction in mice mimicking the dominant skin blistering disease EBS-OgnaPlectin: a cytolinker by designTargeted inactivation of plectin reveals essential function in maintaining the integrity of skin, muscle, and heart cytoarchitectureThe many faces of plectin and plectinopathies: pathology and mechanismsCaspase 8-mediated cleavage of plectin precedes F-actin breakdown in acinar cells during pancreatitisIntermediate filament-associated cytolinker plectin 1c destabilizes microtubules in keratinocytesEpiplakin, a novel member of the Plakin family originally identified as a 450-kDa human epidermal autoantigen. Structure and tissue localization.Characterization of extracellular matrix macromolecules in primary cultures of equine keratinocytesDesmin-related myopathies in mice and man.Plectin deficiency leads to both muscular dystrophy and pyloric atresia in epidermolysis bullosa simplexMyasthenic syndrome caused by plectinopathyPlectin as a prognostic marker in non-metastatic oral squamous cell carcinomaEpidermolysis bullosa simplex associated with pyloric atresia is a novel clinical subtype caused by mutations in the plectin gene (PLEC1)The rod domain is not essential for the function of plectin in maintaining tissue integrityNeurofilament-dependent radial growth of motor axons and axonal organization of neurofilaments does not require the neurofilament heavy subunit (NF-H) or its phosphorylation.Myofiber integrity depends on desmin network targeting to Z-disks and costameres via distinct plectin isoforms.Epidermolysis bullosa simplex: a paradigm for disorders of tissue fragility.Plectin-intermediate filament partnership in skin, skeletal muscle, and peripheral nerve.Molecular architecture and function of the hemidesmosome.Epidermal cell junctions and their regulation by p63 in health and disease.Molecular architecture and function of the hemidesmosome.Costamere proteins and their involvement in myopathic processes.Epidermolysis bullosa simplex with muscular dystrophy. Review of the literature and a case report.Specificity of binding of the plectin actin-binding domain to beta4 integrin.Keith R. Porter Lecture, 1996. Of mice and men: genetic disorders of the cytoskeleton.Integrin alpha 6 beta 4 forms a complex with the cytoskeletal protein HD1 and induces its redistribution in transfected COS-7 cells.Modeling and experimental validation of the binary complex of the plectin actin-binding domain and the first pair of fibronectin type III (FNIII) domains of the beta4 integrin.Role of binding of plectin to the integrin beta4 subunit in the assembly of hemidesmosomes.Linking integrin alpha6beta4-based cell adhesion to the intermediate filament cytoskeleton: direct interaction between the beta4 subunit and plectin at multiple molecular sites.
P2860
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P2860
Defective expression of plectin/HD1 in epidermolysis bullosa simplex with muscular dystrophy
description
1996 nî lūn-bûn
@nan
1996 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
1996 թվականի մայիսին հրատարակված գիտական հոդված
@hy
1996年の論文
@ja
1996年論文
@yue
1996年論文
@zh-hant
1996年論文
@zh-hk
1996年論文
@zh-mo
1996年論文
@zh-tw
1996年论文
@wuu
name
Defective expression of plecti ...... implex with muscular dystrophy
@ast
Defective expression of plecti ...... implex with muscular dystrophy
@en
Defective expression of plecti ...... implex with muscular dystrophy
@nl
type
label
Defective expression of plecti ...... implex with muscular dystrophy
@ast
Defective expression of plecti ...... implex with muscular dystrophy
@en
Defective expression of plecti ...... implex with muscular dystrophy
@nl
prefLabel
Defective expression of plecti ...... implex with muscular dystrophy
@ast
Defective expression of plecti ...... implex with muscular dystrophy
@en
Defective expression of plecti ...... implex with muscular dystrophy
@nl
P2093
P2860
P50
P3181
P356
P1476
Defective expression of plecti ...... implex with muscular dystrophy
@en
P2093
P2860
P304
P3181
P356
10.1172/JCI118671
P407
P577
1996-05-01T00:00:00Z