Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
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Ferlins: regulators of vesicle fusion for auditory neurotransmission, receptor trafficking and membrane repairHomologous recombination mediates functional recovery of dysferlin deficiency following AAV5 gene transferA novel deletion in the GTPase domain of OPA1 causes defects in mitochondrial morphology and distribution, but not in functionMyoferlin regulates vascular endothelial growth factor receptor-2 stability and functionDysferlin regulates cell membrane repair by facilitating injury-triggered acid sphingomyelinase secretion.Recessive mutations in the putative calcium-activated chloride channel Anoctamin 5 cause proximal LGMD2L and distal MMD3 muscular dystrophies.Myoferlin is required for insulin-like growth factor response and muscle growth.Myogenesis in dysferlin-deficient myoblasts is inhibited by an intrinsic inflammatory response.Genetic ablation of complement C3 attenuates muscle pathology in dysferlin-deficient miceMacrophages promote muscle membrane repair and muscle fibre growth and regeneration during modified muscle loading in mice in vivo.The muscle protein dysferlin accumulates in the Alzheimer brain.Myofiber damage precedes macrophage infiltration after in vivo injury in dysferlin-deficient A/J mouse skeletal muscle.Dystrophin deficiency exacerbates skeletal muscle pathology in dysferlin-null mice.Lysosome fusion to the cell membrane is mediated by the dysferlin C2A domain in coronary arterial endothelial cells.Upregulated IL-1β in dysferlin-deficient muscle attenuates regeneration by blunting the response to pro-inflammatory macrophagesEnhanced Muscular Dystrophy from Loss of Dysferlin Is Accompanied by Impaired Annexin A6 Translocation after Sarcolemmal Disruption.The absence of dysferlin induces the expression of functional connexin-based hemichannels in human myotubesDysferlin and muscle membrane repair.Dysferlin deficiency enhances monocyte phagocytosis: a model for the inflammatory onset of limb-girdle muscular dystrophy 2B.Dysferlin stabilizes stress-induced Ca2+ signaling in the transverse tubule membrane.Translational research and therapeutic perspectives in dysferlinopathies.Ferlin proteins in myoblast fusion and muscle growth.Mechanisms of muscle injury, repair, and regeneration.Progress and challenges in diagnosis of dysferlinopathy.Normal myoblast fusion requires myoferlinPolymyositis without Beneficial Response to Steroid Therapy: Should Miyoshi Myopathy be a Differential Diagnosis?Distinct effects of contraction-induced injury in vivo on four different murine models of dysferlinopathy.Diagnosis by protein analysis of dysferlinopathy in two patients mistaken as polymyositis.The clinical course of calpainopathy (LGMD2A) and dysferlinopathy (LGMD2B).Increased non-HDL cholesterol levels cause muscle wasting and ambulatory dysfunction in the mouse model of LGMD2B.Dysferlin is a new marker for leaky brain blood vessels in multiple sclerosis.
P2860
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P2860
Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
2005年论文
@zh
2005年论文
@zh-cn
name
Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
@ast
Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
@en
type
label
Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
@ast
Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
@en
prefLabel
Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
@ast
Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
@en
P2093
P2860
P356
P1476
Ultrastructural changes in dysferlinopathy support defective membrane repair mechanism.
@en
P2093
G Cenacchi
L B De Giorgi
P2860
P304
P356
10.1136/JCP.2004.018978
P407
P577
2005-02-01T00:00:00Z