Limb-girdle muscular dystrophy (LGMD-1C) mutants of caveolin-3 undergo ubiquitination and proteasomal degradation. Treatment with proteasomal inhibitors blocks the dominant negative effect of LGMD-1C mutanta and rescues wild-type caveolin-3.
about
The caveolin proteinsCaveolinopathies: from the biology of caveolin-3 to human diseasesDefective interactions of protein partner with ion channels and transporters as alternative mechanisms of membrane channelopathiesL166P mutant DJ-1, causative for recessive Parkinson's disease, is degraded through the ubiquitin-proteasome systemModulation of myoblast fusion by caveolin-3 in dystrophic skeletal muscle cells: implications for Duchenne muscular dystrophy and limb-girdle muscular dystrophy-1CSpatial and temporal regulation of GLUT4 translocation by flotillin-1 and caveolin-3 in skeletal muscle cellsCaveolin-3 null mice show a loss of caveolae, changes in the microdomain distribution of the dystrophin-glycoprotein complex, and t-tubule abnormalitiesCholesterol and fatty acids regulate dynamic caveolin trafficking through the Golgi complex and between the cell surface and lipid bodies.The tetraspan protein EMP2 regulates expression of caveolin-1.Expression of caveolin-1 induces premature cellular senescence in primary cultures of murine fibroblasts.Identification and functional analysis of a new putative caveolin-3 variant found in a patient with sudden unexplained deathInteraction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.Defining a new paradigm for human arrhythmia syndromes: phenotypic manifestations of gene mutations in ion channel- and transporter-associated proteins.Proteasomal inhibition restores biological function of mis-sense mutated dysferlin in patient-derived muscle cells.TRIM72 is required for effective repair of alveolar epithelial cell wounding.Caveolin-1 is ubiquitinated and targeted to intralumenal vesicles in endolysosomes for degradation.Caveolin-deficient mice: insights into caveolar function human diseaseProteasome inhibitor (MG-132) treatment of mdx mice rescues the expression and membrane localization of dystrophin and dystrophin-associated proteinsRippling muscle disease and facioscapulohumeral dystrophy-like phenotype in a patient carrying a heterozygous CAV3 T78M mutation and a D4Z4 partial deletion: Further evidence for "double trouble" overlapping syndromesUbiquitination of the N-terminal region of caveolin-1 regulates endosomal sorting by the VCP/p97 AAA-ATPaseInhibition of proteasome activity promotes the correct localization of disease-causing alpha-sarcoglycan mutants in HEK-293 cells constitutively expressing beta-, gamma-, and delta-sarcoglycan.Overexpression of caveolin-1 is sufficient to phenocopy the behavior of a disease-associated mutantMechanisms of human arrhythmia syndromes: abnormal cardiac macromolecular interactions.Caveolin-1 (P132L), a common breast cancer mutation, confers mammary cell invasiveness and defines a novel stem cell/metastasis-associated gene signature.Myoferlin is critical for endocytosis in endothelial cells.Caveolins and cavins in the trafficking, maturation, and degradation of caveolae: implications for cell physiology.ABC of multifaceted dystrophin glycoprotein complex (DGC).Oxidative stress induces premature senescence by stimulating caveolin-1 gene transcription through p38 mitogen-activated protein kinase/Sp1-mediated activation of two GC-rich promoter elements.Traffic jams II: an update of diseases of intracellular transport.The metastatic suppressor NDRG1 inhibits EMT, migration and invasion through interaction and promotion of caveolin-1 ubiquitylation in human colorectal cancer cells.Revisiting caveolin trafficking: the end of the caveosome.The Caveolin-3 G56S sequence variant of unknown significance: Muscle biopsy findings and functional cell biological analysis.Potential of caveolae in the therapy of cardiovascular and neurological diseases.Consequences of a novel caveolin-3 mutation in a large German family.Caveolin-3 T78M and T78K missense mutations lead to different phenotypes in vivo and in vitro.A disease-associated frameshift mutation in caveolin-1 disrupts caveolae formation and function through introduction of a de novo ER retention signal.Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice.Identification of Novel Antisense-Mediated Exon Skipping Targets in DYSF for Therapeutic Treatment of Dysferlinopathy
P2860
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P2860
Limb-girdle muscular dystrophy (LGMD-1C) mutants of caveolin-3 undergo ubiquitination and proteasomal degradation. Treatment with proteasomal inhibitors blocks the dominant negative effect of LGMD-1C mutanta and rescues wild-type caveolin-3.
description
2000 nî lūn-bûn
@nan
2000年の論文
@ja
2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
@zh-tw
2000年论文
@wuu
2000年论文
@zh
2000年论文
@zh-cn
name
Limb-girdle muscular dystrophy ...... rescues wild-type caveolin-3.
@en
Limb-girdle muscular dystrophy
@nl
type
label
Limb-girdle muscular dystrophy ...... rescues wild-type caveolin-3.
@en
Limb-girdle muscular dystrophy
@nl
prefLabel
Limb-girdle muscular dystrophy ...... rescues wild-type caveolin-3.
@en
Limb-girdle muscular dystrophy
@nl
P2093
P2860
P356
P1476
Limb-girdle muscular dystrophy ...... rescues wild-type caveolin-3.
@en
P2093
D B Bregman
F Galbiati
M P Lisanti
P2860
P304
37702-37711
P356
10.1074/JBC.M006657200
P407
P577
2000-12-01T00:00:00Z