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Emilin1 links TGF-beta maturation to blood pressure homeostasisCyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathiesElastic fiber proteins in the glomerular mesangium in vivo and in cell cultureEMILIN-1 deficiency induces elastogenesis and vascular cell defectsCationic PMMA nanoparticles bind and deliver antisense oligoribonucleotides allowing restoration of dystrophin expression in the mdx mouseChanges in muscle cell metabolism and mechanotransduction are associated with myopathic phenotype in a mouse model of collagen VI deficiency.Muscle proteomics reveals novel insights into the pathophysiological mechanisms of collagen VI myopathies.NG2/CSPG4-collagen type VI interplays putatively involved in the microenvironmental control of tumour engraftment and local expansion.Deep RNA profiling identified CLOCK and molecular clock genes as pathophysiological signatures in collagen VI myopathyBiodistribution studies of polymeric nanoparticles for drug delivery in mice.Persistent dystrophin protein restoration 90 days after a course of intraperitoneally administered naked 2'OMePS AON and ZM2 NP-AON complexes in mdx mice.Cyclosporin A Promotes in vivo Myogenic Response in Collagen VI-Deficient Myopathic Mice.Biodistribution and molecular studies on orally administered nanoparticle-AON complexes encapsulated with alginate aiming at inducing dystrophin rescue in mdx mice.The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1-/- myopathic mice.Analysis of beta cell proliferation dynamics in zebrafish.Oxidative stress by monoamine oxidases is causally involved in myofiber damage in muscular dystrophy.Mechanisms of transcriptional activation of the col6a1 gene during Schwann cell differentiation.Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency.pUBEX/pUBSEX: a versatile expression vector system for production of fusion and nonfusion proteins in Escherichia coli.NIM811, a cyclophilin inhibitor without immunosuppressive activity, is beneficial in collagen VI congenital muscular dystrophy models.Overlapping, complementary and site-specific expression pattern of genes of the EMILIN/Multimerin family.An enhancer required for transcription of the Col6a1 gene in muscle connective tissue is induced by signals released from muscle cells.Emilin genes are duplicated and dynamically expressed during zebrafish embryonic development.The ablation of the matricellular protein EMILIN2 causes defective vascularization due to impaired EGFR-dependent IL-8 production affecting tumor growth.Collagen VI deficiency affects the organization of fibronectin in the extracellular matrix of cultured fibroblastsLoss of mitochondrial calcium uniporter rewires skeletal muscle metabolism and substrate preference.Expression of the EMILIN-1 gene during mouse developmentAnalysis of regulatory regions of Emilin1 gene and their combinatorial contribution to tissue-specific transcription
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description
hulumtuese
@sq
researcher
@en
wetenschapper
@nl
հետազոտող
@hy
name
Paola Braghetta
@ast
Paola Braghetta
@en
Paola Braghetta
@es
Paola Braghetta
@nl
type
label
Paola Braghetta
@ast
Paola Braghetta
@en
Paola Braghetta
@es
Paola Braghetta
@nl
prefLabel
Paola Braghetta
@ast
Paola Braghetta
@en
Paola Braghetta
@es
Paola Braghetta
@nl
P106
P1153
6603607272
P21
P31
P496
0000-0003-2547-8679