Proteasomal inhibition restores biological function of mis-sense mutated dysferlin in patient-derived muscle cells.
about
Dysferlin-peptides reallocate mutated dysferlin thereby restoring functionThe intriguing regulators of muscle mass in sarcopenia and muscular dystrophy.Modular dispensability of dysferlin C2 domains reveals rational design for mini-dysferlin molecules.Roscovitine is a proteostasis regulator that corrects the trafficking defect of F508del-CFTR by a CDK-independent mechanismDegradation of gamma secretase activating protein by the ubiquitin-proteasome pathway.The absence of dysferlin induces the expression of functional connexin-based hemichannels in human myotubesDisease-proportional proteasomal degradation of missense dystrophins1α,25(OH)(2)-Vitamin D3 increases dysferlin expression in vitro and in a human clinical trial.Cell healing: Calcium, repair and regeneration.The Clinical Outcome Study for dysferlinopathy: An international multicenter study.Therapeutic potential of proteasome inhibitors in congenital erythropoietic porphyria.The Classification, Natural History and Treatment of the Limb Girdle Muscular DystrophiesDysferlin-deficient muscular dystrophy and innate immune activation.Identification of splicing defects caused by mutations in the dysferlin gene.A novel congenital disorder of glycosylation type without central nervous system involvement caused by mutations in the phosphoglucomutase 1 gene.RETRACTED: Proteasome inhibitors increase missense mutated dysferlin in patients with muscular dystrophy.The ubiquitin ligase tripartite-motif-protein 32 is induced in Duchenne muscular dystrophy.Muscle atrophy, ubiquitin-proteasome, and autophagic pathways in dysferlinopathy.
P2860
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P2860
Proteasomal inhibition restores biological function of mis-sense mutated dysferlin in patient-derived muscle cells.
description
2012 nî lūn-bûn
@nan
2012 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2012 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
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
name
Proteasomal inhibition restore ...... patient-derived muscle cells.
@ast
Proteasomal inhibition restore ...... patient-derived muscle cells.
@en
Proteasomal inhibition restore ...... patient-derived muscle cells.
@nl
type
label
Proteasomal inhibition restore ...... patient-derived muscle cells.
@ast
Proteasomal inhibition restore ...... patient-derived muscle cells.
@en
Proteasomal inhibition restore ...... patient-derived muscle cells.
@nl
prefLabel
Proteasomal inhibition restore ...... patient-derived muscle cells.
@ast
Proteasomal inhibition restore ...... patient-derived muscle cells.
@en
Proteasomal inhibition restore ...... patient-derived muscle cells.
@nl
P2093
P2860
P356
P1476
Proteasomal inhibition restore ...... patient-derived muscle cells.
@en
P2093
Bilal A Azakir
Jochen Kinter
Michael Sinnreich
Sabrina Di Fulvio
P2860
P304
10344-10354
P356
10.1074/JBC.M111.329078
P407
P577
2012-02-08T00:00:00Z