Dysferlin deficiency shows compensatory induction of Rab27A/Slp2a that may contribute to inflammatory onset.
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Crystal structures of the human Dysferlin inner DysF domainUpregulation of the Rab27a-dependent trafficking and secretory mechanisms improves lysosomal transport, alleviates endoplasmic reticulum stress, and reduces lysosome overload in cystinosis.Dysferlin regulates cell membrane repair by facilitating injury-triggered acid sphingomyelinase secretion.Extensive mononuclear infiltration and myogenesis characterize recovery of dysferlin-null skeletal muscle from contraction-induced injuries.Autoantibody profiles in two patients with non-autoimmune muscle disease implicate a role for gliadin autoreactivity.Limb-girdle and congenital muscular dystrophies: current diagnostics, management, and emerging technologies.Statistical Test of Expression Pattern (STEPath): a new strategy to integrate gene expression data with genomic information in individual and meta-analysis studies.Inflammasome up-regulation and activation in dysferlin-deficient skeletal muscle.New aspects on patients affected by dysferlin deficient muscular dystrophyNon-invasive optical imaging of muscle pathology in mdx mice using cathepsin caged near-infrared imagingActin cytoskeleton remodeling by the alternatively spliced isoform of PDLIM4/RIL protein.Myofiber damage precedes macrophage infiltration after in vivo injury in dysferlin-deficient A/J mouse skeletal muscle.Inhibition of inflammation with celastrol fails to improve muscle function in dysferlin-deficient A/J miceElusive sources of variability of dystrophin rescue by exon skippingPlacental dysferlin expression is reduced in severe preeclampsia.Genetic manipulation of dysferlin expression in skeletal muscle: novel insights into muscular dystrophy.Translational research and therapeutic perspectives in dysferlinopathies.Rab27 effectors, pleiotropic regulators in secretory pathways.Dysferlin aggregation in limb-girdle muscular dystrophy type 2B/Miyoshi Myopathy necessitates mutational screen for diagnosis [corrected].Increased non-HDL cholesterol levels cause muscle wasting and ambulatory dysfunction in the mouse model of LGMD2B.Annexin A2 links poor myofiber repair with inflammation and adipogenic replacement of the injured muscle.Role of thrombospondin 1 in macrophage inflammation in dysferlin myopathy.Diltiazem improves contractile properties of skeletal muscle in dysferlin-deficient BLAJ mice, but does not reduce contraction-induced muscle damage.
P2860
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P2860
Dysferlin deficiency shows compensatory induction of Rab27A/Slp2a that may contribute to inflammatory onset.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
Dysferlin deficiency shows com ...... tribute to inflammatory onset.
@en
type
label
Dysferlin deficiency shows com ...... tribute to inflammatory onset.
@en
prefLabel
Dysferlin deficiency shows com ...... tribute to inflammatory onset.
@en
P2093
P2860
P50
P1476
Dysferlin deficiency shows com ...... ntribute to inflammatory onset
@en
P2093
Eric P Hoffman
Gustavo A Nader
Rumaisa Bashir
Susan Knoblach
P2860
P304
P356
10.2353/AJPATH.2008.080098
P407
P577
2008-10-02T00:00:00Z