P. Braghetta

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AMBRA1 Controls Regulatory T-Cell Differentiation and Homeostasis Upstream of the FOXO3-FOXP3 Axis.

D. Centonze, S. Campello, G. Botti, 2018, Developmental cell.

Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency

C. Reggiani, P. Braghetta, P. Bonaldo, 2003, Nature Genetics.

Biodistribution and Molecular Studies on Orally Administered Nanoparticle-AON Complexes Encapsulated with Alginate Aiming at Inducing Dystrophin Rescue in mdx Mice

M. Falzarano, A. Ferlini, N. Maraldi, 2013, BioMed research international.

A novel murine model for arrhythmogenic cardiomyopathy points to a pathogenic role of Wnt signalling and miRNA dysregulation

G. Lembo, P. Braghetta, P. Bonaldo, 2018, Cardiovascular research.

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

J. Luzio, P. Braghetta, G. Banting, 1991, Gene.

Electrospun Structures Made of a Hydrolyzed Keratin-Based Biomaterial for Development of in vitro Tissue Models

P. Braghetta, G. Vozzi, C. De Maria, 2019, Front. Bioeng. Biotechnol..

Pathogenic Potential of Hic1-Expressing Cardiac Stromal Progenitors.

F. Rossi, V. Korinek, P. Braghetta, 2020, Cell stem cell.

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

A. Ferlini, N. Maraldi, P. Braghetta, 2008, Proceedings of the National Academy of Sciences.

FGF signaling deregulation is associated with early developmental skeletal defects in animal models for mucopolysaccharidosis type II (MPSII)

R. Tomanin, M. Filocamo, P. Braghetta, 2018, Human molecular genetics.

Cell type-specific transcription of the alpha1(VI) collagen gene. Role of the AP1 binding site and of the core promoter.

P. Braghetta, S. Piccolo, D. Volpin, 1999, The Journal of biological chemistry.

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

P. Braghetta, P. Bonaldo, D. Volpin, 2001, Mechanisms of Development.

Gelatin-genipin-based biomaterials for skeletal muscle tissue engineering.

P. Pinton, P. Braghetta, P. Bonaldo, 2018, Journal of biomedical materials research. Part B, Applied biomaterials.

Lentiviral-mediated RNAi in vivo silencing of Col6a1, a gene with complex tissue specific expression pattern.

P. Braghetta, P. Bonaldo, M. Curtarello, 2009, Journal of biotechnology.

Mapping Wnt/β-catenin signaling during mouse development and in colorectal tumors

S. Dupont, P. Braghetta, V. Broccoli, 2003, Proceedings of the National Academy of Sciences of the United States of America.

Distinct regions control transcriptional activation of the alpha1(VI) collagen promoter in different tissues of transgenic mice

P. Braghetta, P. Bonaldo, S. Piccolo, 1996, The Journal of cell biology.

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

P. Braghetta, P. Bonaldo, D. Volpin, 2004, Matrix biology : journal of the International Society for Matrix Biology.

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

P. Braghetta, P. Bonaldo, F. Gattazzo, 2014, Front. Aging Neurosci..

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

P. Braghetta, P. Bonaldo, C. Baldari, 2009, British journal of pharmacology.

Changes in Muscle Cell Metabolism and Mechanotransduction Are Associated with Myopathic Phenotype in a Mouse Model of Collagen VI Deficiency

P. Braghetta, P. Bonaldo, C. Gelfi, 2013, PloS one.

Identification, sequencing and expression of an integral membrane protein of the trans-Golgi network (TGN38).

K. Howell, J. Luzio, P. Braghetta, 1990, The Biochemical journal.

A new recombinant DNA strategy for the molecular cloning of rare membrane proteins.

J. Luzio, P. Braghetta, G. Banting, 1990, The Biochemical journal.

Collagen VI Null Mice as a Model for Early Onset Muscle Decline in Aging

P. Braghetta, P. Bonaldo, C. Gelfi, 2017, Front. Mol. Neurosci..

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

N. Maraldi, P. Braghetta, P. Bonaldo, 2014, Human molecular genetics.

Loss of Mitochondrial Calcium Uniporter rewires skeletal muscle metabolism and substrate preference

C. Mammucari, R. Rizzuto, P. Braghetta, 2018, Cell Death & Differentiation.

Ambra1 deficiency impairs mitophagy in skeletal muscle

F. Cecconi, E. Trevisson, F. Strappazzon, 2022, Journal of cachexia, sarcopenia and muscle.

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

C. Reggiani, L. Toniolo, P. Braghetta, 2010, Human molecular genetics.

Lack of COL6/collagen VI causes megakaryocyte dysfunction by impairing autophagy and inducing apoptosis

A. Balduini, E. Pegoraro, P. Braghetta, 2022, Autophagy.

Extracellular Collagen VI Has Prosurvival and Autophagy Instructive Properties in Mouse Fibroblasts

P. Braghetta, M. Chrisam, P. Bonaldo, 2018, Front. Physiol..

Role of adiponectin in the metabolism of skeletal muscles in collagen VI–related myopathies

A. Modesti, P. Braghetta, M. Chrisam, 2019, Journal of Molecular Medicine.

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

E. Bertini, N. Maraldi, C. Capanni, 2001, Matrix biology : journal of the International Society for Matrix Biology.

Analysis of beta cell proliferation dynamics in zebrafish.

P. Braghetta, F. Argenton, E. Moro, 2009, Developmental biology.

Ablation of collagen VI leads to the release of platelets with altered function

A. Balduini, E. Pegoraro, F. Moccia, 2021, Blood advances.

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

F. Muntoni, Hanns Lochmüller, A. Ferlini, 2014, Journal of proteome research.

Preclinical PK and PD Studies on 2′-O-Methyl-phosphorothioate RNA Antisense Oligonucleotides in the mdx Mouse Model

G. van Ommen, A. Ferlini, Annemieke Aartsma-Rus, 2010, Molecular therapy : the journal of the American Society of Gene Therapy.

Pathogenic potential of Hic1 expressing cardiac stromal progenitors

F. Rossi, P. Braghetta, K. Macleod, 2019, bioRxiv.

Collagen VI deficiency causes behavioral abnormalities and cortical dopaminergic dysfunction

C. Semenza, E. Pegoraro, P. Braghetta, 2022, Disease models & mechanisms.

Emilin-2 is a component of bone marrow extracellular matrix regulating mesenchymal stem cell differentiation and hematopoietic progenitors

P. Braghetta, P. Bonaldo, L. Persano, 2022, Stem Cell Research & Therapy.

Program and Book of the 15th Interuniversity Institute of Myology Meeting - Assisi (Italy), 2018

W. Świȩszkowski, G. Forte, A. Musarò, 2018, European journal of translational myology.

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

U. Schlötzer-Schrehardt, P. Braghetta, A. Hartner, 2000, Kidney international.

An ultrastructural approach to the adaptive role of the cell wall in the intertidal algaFucus virsoides

P. Braghetta, P. Mariani, C. Tolomio, 1985, Protoplasma.

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

I. Jurisica, F. Bucciotti, P. Braghetta, 2018, Oncogene.

Collagen VI deficiency induces early onset myopathy in the mouse: an animal model for Bethlem myopathy.

P. Braghetta, P. Bonaldo, S. Piccolo, 1998, Human molecular genetics.

EMILIN1 deficiency causes arterial tortuosity with osteopenia and connects impaired elastogenesis with defective collagen fibrillogenesis.

Takako Sasaki, S. Ellard, Stacey L. Cole, 2022, American journal of human genetics.

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

Luciano Merlini, Paola Braghetta, Alessandra Ferlini, 2009, Molecular therapy : the journal of the American Society of Gene Therapy.

Hmga2 promoter analysis in transgenic mice.

K. Chada, Jun Liu, A. Rustighi, 2003, Biochemical and biophysical research communications.

Emilin1 Links TGF-β Maturation to Blood Pressure Homeostasis

S. Dupont, G. Lembo, P. Braghetta, 2006, Cell.

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

Pier Andrea Nicolosi, P. Picci, P. Braghetta, 2013, Journal of molecular cell biology.

Analysis of Regulatory Regions of Emilin1 Gene and Their Combinatorial Contribution to Tissue-specific Transcription*

P. Braghetta, P. Bonaldo, A. Colombatti, 2005, Journal of Biological Chemistry.

F-actin dynamics regulates mammalian organ growth and cell fate maintenance.

S. Bicciato, K. Franze, S. Dupont, 2019, Journal of hepatology.

Analysis of Transcription of the Col6a1 Gene in a Specific Set of Tissues Suggests a New Variant of Enhancer Region*

P. Braghetta, D. Volpin, G. Bressan, 2000, The Journal of Biological Chemistry.

Wnt/β-catenin pathway in arrhythmogenic cardiomyopathy

P. Braghetta, L. D. de Windt, M. Calore, 2017, Oncotarget.

Collagen VI in healthy and diseased nervous system

P. Braghetta, P. Bonaldo, M. Cescon, 2018, Disease Models & Mechanisms.

EMILIN-1 Deficiency Induces Elastogenesis and Vascular Cell Defects

P. Braghetta, P. Bonaldo, A. Colombatti, 2004, Molecular and Cellular Biology.

Isolation of embryonic stem (ES) cells in media supplemented with recombinant leukemia inhibitory factor (LIF).

R. Williams, P. Braghetta, S. Pease, 1990, Developmental biology.

First steps to define murine amniotic fluid stem cell microenvironment

M. Pozzobon, P. Braghetta, P. Bonaldo, 2016, Scientific Reports.

Multimerin-2 maintains vascular stability and permeability.

F. Bucciotti, R. Iozzo, P. Braghetta, 2020, Matrix biology : journal of the International Society for Matrix Biology.

Tissue-specific expression of promoter regions of the alpha1(VI) collagen gene in cell cultures and transgenic mice.

P. Braghetta, P. Bonaldo, S. Piccolo, 1997, European journal of biochemistry.

Persistent Dystrophin Protein Restoration 90 Days after a Course of Intraperitoneally Administered Naked 2′OMePS AON and ZM2 NP-AON Complexes in mdx Mice

A. Ferlini, P. Braghetta, P. Bonaldo, 2012, Journal of biomedicine & biotechnology.

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

P. Braghetta, P. Bonaldo, D. Volpin, 2008, Experimental cell research.

Cell wall ultrastructure and cation localization in some benthic marine algae

P. Braghetta, B. Baldan, P. Mariani, 1990 .

Emerging Perspectives on the Rare Tubulopathy Dent Disease: Is Glomerular Damage a Direct Consequence of ClC-5 Dysfunction?

F. Anglani, P. Braghetta, M. Ceol, 2023, International journal of molecular sciences.

Fra-2-expressing macrophages promote lung fibrosis in mice.

E. Wagner, L. Paz-Ares, D. Megías, 2019, The Journal of clinical investigation.

Targeting Interleukin‐1&bgr; Protects from Aortic Aneurysms Induced by Disrupted Transforming Growth Factor &bgr; Signaling

G. Lembo, P. Braghetta, G. Bressan, 2017, Immunity.

Loss of EMILIN-1 Enhances Arteriolar Myogenic Tone Through TGF-&bgr; (Transforming Growth Factor-&bgr;)–Dependent Transactivation of EGFR (Epidermal Growth Factor Receptor) and Is Relevant for Hypertension in Mice and Humans

R. Rizzuto, G. Lembo, P. Braghetta, 2018, Arteriosclerosis, thrombosis, and vascular biology.

The Polyphenol Pterostilbene Ameliorates the Myopathic Phenotype of Collagen VI Deficient Mice via Autophagy Induction

P. Braghetta, M. Chrisam, P. Bonaldo, 2020, Frontiers in Cell and Developmental Biology.

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*

M. Paulsson, P. Braghetta, P. Bonaldo, 2012, The Journal of Biological Chemistry.

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

G. Pesole, E. Bertini, K. Bushby, 2016, Journal of Cell Science.

Intracellular targetting signals of polymeric immunoglobulin receptors are highly conserved between species

J. Luzio, P. Braghetta, G. Banting, 1989, FEBS letters.

Preclinical PK and PD Studies on 2'-O-Methyl-phosphorothioate RNA Antisense Oligonucleotides in the mdx Mouse Model.

G. van Ommen, A. Ferlini, Annemieke Aartsma-Rus, 2010, Molecular therapy : the journal of the American Society of Gene Therapy.

Biodistribution studies of polymeric nanoparticles for drug delivery in mice.

M. Falzarano, A. Ferlini, P. Braghetta, 2014, Human gene therapy.

Dystrophin restoration in skeletal, heart and skin arrector pili smooth muscle of mdx mice by ZM2 NP–AON complexes

A. Ferlini, N. Maraldi, P. Braghetta, 2010, Gene Therapy.

Vascular Smooth Muscle Emilin-1 Is a Regulator of Arteriolar Myogenic Response and Blood Pressure

G. Lembo, P. Braghetta, A. Colombatti, 2012, Arteriosclerosis, thrombosis, and vascular biology.

Collagen VI sustains cell stemness and chemotherapy resistance in glioblastoma

P. Braghetta, P. Bonaldo, A. Cani, 2023, Cellular and Molecular Life Sciences.

Emilin genes are duplicated and dynamically expressed during zebrafish embryonic development

P. Braghetta, P. Bonaldo, D. Volpin, 2008, Developmental dynamics : an official publication of the American Association of Anatomists.

Expression of the EMILIN-1 gene during mouse development.

P. Braghetta, P. Bonaldo, D. Volpin, 2002, Matrix biology : journal of the International Society for Matrix Biology.

Collagen VI sustains cell stemness and chemotherapy resistance in glioblastoma

P. Braghetta, P. Bonaldo, A. Cani, 2023, Cellular and Molecular Life Sciences.

Fra-2-expressing macrophages promote lung fibrosis in mice.

E. Wagner, L. Paz-Ares, D. Megías, 2019, The Journal of clinical investigation.

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

A. Ferlini, N. Maraldi, P. Braghetta, 2008, Proceedings of the National Academy of Sciences.

Role of adiponectin in the metabolism of skeletal muscles in collagen VI–related myopathies

A. Modesti, P. Braghetta, M. Chrisam, 2019, Journal of molecular medicine.

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

C. Reggiani, L. Toniolo, P. Braghetta, 2010, Human molecular genetics.

Vascular Smooth Muscle Emilin-1 Is a Regulator of Arteriolar Myogenic Response and Blood Pressure

G. Lembo, P. Braghetta, A. Colombatti, 2012, Arteriosclerosis, thrombosis, and vascular biology.

Cell Type-specific Transcription of the α1(VI) Collagen Gene

P. Braghetta, D. Volpin, G. Bressan, 1999, The Journal of Biological Chemistry.

M. Paulsson, P. Braghetta, P. Bonaldo, 2012, The Journal of Biological Chemistry.

GBA1 inactivation in oligodendrocytes affects myelination and induces neurodegenerative hallmarks and lipid dyshomeostasis in mice

P. Braghetta, M. Moriggi, F. Papaleo, 2024, Molecular neurodegeneration.

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

C. Gargioli, P. Braghetta, P. Bonaldo, 2024, International journal of molecular sciences.