List of works - Paola Braghetta

An enhancer required for transcription of the Col6a1 gene in muscle connective tissue is induced by signals released from muscle cells.

scientific article published on 19 August 2008

Analysis of beta cell proliferation dynamics in zebrafish.

scientific article published on 3 June 2009

Analysis of regulatory regions of Emilin1 gene and their combinatorial contribution to tissue-specific transcription

scientific article published on 10 February 2005

Biodistribution and molecular studies on orally administered nanoparticle-AON complexes encapsulated with alginate aiming at inducing dystrophin rescue in mdx mice

scientific article

Biodistribution studies of polymeric nanoparticles for drug delivery in mice.

scientific article published on 22 September 2014

Cationic PMMA nanoparticles bind and deliver antisense oligoribonucleotides allowing restoration of dystrophin expression in the mdx mouse

scientific article

Changes in muscle cell metabolism and mechanotransduction are associated with myopathic phenotype in a mouse model of collagen VI deficiency.

scientific article

Characterization of Proteome Changes in Aged and Collagen VI-Deficient Human Pericyte Cultures

scientific article published in 2024

Collagen VI deficiency affects the organization of fibronectin in the extracellular matrix of cultured fibroblasts

article

Cyclosporin A Promotes in vivo Myogenic Response in Collagen VI-Deficient Myopathic Mice.

scientific article published on 15 September 2014

Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies

scientific article

Deep RNA profiling identified CLOCK and molecular clock genes as pathophysiological signatures in collagen VI myopathy

scientific article published on 4 March 2016

EMILIN-1 deficiency induces elastogenesis and vascular cell defects

scientific journal article

EMILIN-3, Peculiar Member of Elastin Microfibril Interface-located Protein (EMILIN) Family, Has Distinct Expression Pattern, Forms Oligomeric Assemblies, and Serves as Transforming Growth Factor β (TGF-β) Antagonist ⬇️

scientific article published on February 10, 2012

Elastic fiber proteins in the glomerular mesangium in vivo and in cell culture

scientific journal article

Emilin genes are duplicated and dynamically expressed during zebrafish embryonic development.

scientific article published in January 2008

Emilin1 links TGF-beta maturation to blood pressure homeostasis

scientific article

Expression of the EMILIN-1 gene during mouse development

scientific article published on 01 November 2002

Loss of mitochondrial calcium uniporter rewires skeletal muscle metabolism and substrate preference.

scientific article published on 19 September 2018

Mechanisms of transcriptional activation of the col6a1 gene during Schwann cell differentiation.

scientific article published in April 2001

Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency.

scientific article published on 16 November 2003

Muscle proteomics reveals novel insights into the pathophysiological mechanisms of collagen VI myopathies

scientific article

NG2/CSPG4-collagen type VI interplays putatively involved in the microenvironmental control of tumour engraftment and local expansion.

scientific article published on 3 April 2013

NIM811, a cyclophilin inhibitor without immunosuppressive activity, is beneficial in collagen VI congenital muscular dystrophy models.

scientific article published on 22 May 2014

Overlapping, complementary and site-specific expression pattern of genes of the EMILIN/Multimerin family.

scientific article published in January 2004

Oxidative stress by monoamine oxidases is causally involved in myofiber damage in muscular dystrophy

scientific article published on 17 August 2010

Persistent dystrophin protein restoration 90 days after a course of intraperitoneally administered naked 2'OMePS AON and ZM2 NP-AON complexes in mdx mice

scientific article published on 2 October 2012

The ablation of the matricellular protein EMILIN2 causes defective vascularization due to impaired EGFR-dependent IL-8 production affecting tumor growth.

scientific article published on 27 February 2018

The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1-/- myopathic mice.

scientific article published on 10 June 2009

pUBEX/pUBSEX: a versatile expression vector system for production of fusion and nonfusion proteins in Escherichia coli.

scientific article

List of works by Paola Braghetta

An enhancer required for transcription of the Col6a1 gene in muscle connective tissue is induced by signals released from muscle cells.

Analysis of beta cell proliferation dynamics in zebrafish.

Analysis of regulatory regions of Emilin1 gene and their combinatorial contribution to tissue-specific transcription

Biodistribution and molecular studies on orally administered nanoparticle-AON complexes encapsulated with alginate aiming at inducing dystrophin rescue in mdx mice

Biodistribution studies of polymeric nanoparticles for drug delivery in mice.

Cationic PMMA nanoparticles bind and deliver antisense oligoribonucleotides allowing restoration of dystrophin expression in the mdx mouse

Changes in muscle cell metabolism and mechanotransduction are associated with myopathic phenotype in a mouse model of collagen VI deficiency.

Characterization of Proteome Changes in Aged and Collagen VI-Deficient Human Pericyte Cultures

Collagen VI deficiency affects the organization of fibronectin in the extracellular matrix of cultured fibroblasts

Cyclosporin A Promotes in vivo Myogenic Response in Collagen VI-Deficient Myopathic Mice.

Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies

Deep RNA profiling identified CLOCK and molecular clock genes as pathophysiological signatures in collagen VI myopathy

EMILIN-1 deficiency induces elastogenesis and vascular cell defects

EMILIN-3, Peculiar Member of Elastin Microfibril Interface-located Protein (EMILIN) Family, Has Distinct Expression Pattern, Forms Oligomeric Assemblies, and Serves as Transforming Growth Factor β (TGF-β) Antagonist ⬇️

Elastic fiber proteins in the glomerular mesangium in vivo and in cell culture

Emilin genes are duplicated and dynamically expressed during zebrafish embryonic development.

Emilin1 links TGF-beta maturation to blood pressure homeostasis

Expression of the EMILIN-1 gene during mouse development

Loss of mitochondrial calcium uniporter rewires skeletal muscle metabolism and substrate preference.

Mechanisms of transcriptional activation of the col6a1 gene during Schwann cell differentiation.

Mitochondrial dysfunction and apoptosis in myopathic mice with 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.

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.

Oxidative stress by monoamine oxidases is causally involved in myofiber damage in muscular dystrophy

Persistent dystrophin protein restoration 90 days after a course of intraperitoneally administered naked 2'OMePS AON and ZM2 NP-AON complexes in mdx mice

The ablation of the matricellular protein EMILIN2 causes defective vascularization due to impaired EGFR-dependent IL-8 production affecting tumor growth.

The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1-/- myopathic mice.

pUBEX/pUBSEX: a versatile expression vector system for production of fusion and nonfusion proteins in Escherichia coli.