P. Bonaldo

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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.

Mitochondrial dysfunction in the pathogenesis of Ullrich congenital muscular dystrophy and prospective therapy with cyclosporins

A. Ferlini, N. Maraldi, P. Bonaldo, 2007, Proceedings of the National Academy of Sciences.

Collagen VI at a glance

P. Bonaldo, M. Cescon, F. Gattazzo, 2015, Journal of Cell Science.

Collagen VI in cancer and its biological mechanisms.

P. Bonaldo, M. Cescon, Peiwen Chen, 2013, Trends in molecular medicine.

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.

Guidelines for the use and interpretation of assays for monitoring autophagy

Robert Clarke, Hong Jiang, Hiroyuki Ishida, 2012 .

Guidelines for the use and interpretation of assays formonitoring autophagy (3rd edition)

Sangeeta Khare, Xin Qi, Ying Wang, 2016 .

Adipocyte-derived collagen VI affects early mammary tumor progression in vivo, demonstrating a critical interaction in the tumor/stroma microenvironment.

E. Petricoin, L. Liotta, B. Trock, 2005, The Journal of clinical investigation.

Zebrafish ambra1a and ambra1b Silencing Affect Heart Development.

F. Cecconi, F. Nazio, M. Chrisam, 2020, Zebrafish.

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.

Biosynthesis of chick type VI collagen. II. Processing and secretion in fibroblasts and smooth muscle cells.

P. Bonaldo, A. Colombatti, 1987, The Journal of biological chemistry.

The superfamily of proteins with von Willebrand factor type A-like domains: one theme common to components of extracellular matrix, hemostasis, cellular adhesion, and defense mechanisms.

P. Bonaldo, A. Colombatti, 1991, Blood.

Efficient gene trap screening for novel developmental genes using IRES beta geo vector and in vitro preselection.

P. Gruss, K. Chowdhury, P. Bonaldo, 1998, Experimental cell research.

Role of the ECM in notochord formation, function and disease

P. Bonaldo, Diana Corallo, Valeria Trapani, 2017, Journal of Cell Science.

Emilin3 is required for notochord sheath integrity and interacts with Scube2 to regulate notochord-derived Hedgehog signals

P. Bonaldo, Diana Corallo, F. Argenton, 2013, Development.

The superfamily of proteins with von Willebrand factor type A-like domains: one theme common to components of extracellular matrix, hemostasis, cellular adhesion, and defense mechanisms.

P. Bonaldo, A. Colombatti, 1991, Blood.

Collagen VI myopathies: from the animal model to the clinical trial.

N. Maraldi, P. Bonaldo, A. Zamparelli, 2009, Advances in enzyme regulation.

Altered threshold of the mitochondrial permeability transition pore in Ullrich congenital muscular dystrophy.

P. Bonaldo, P. Bernardi, A. Angelin, 2008, Biochimica et biophysica acta.

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.

Multiple binding reactivities of an IgG1 mouse monoclonal antibody raised against the extracellular matrix glycoprotein Gp 115.

P. Bonaldo, A. Colombatti, D. Volpin, 1987, Hybridoma.

Collagens and Muscle Diseases: A Focus on Collagen VI

P. Bonaldo, M. Cescon, Valentina Tonelotto, 2021 .

Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degeneration

N. Maraldi, M. Sandri, E. Bertaggia, 2010, Nature Medicine.

Misregulation of autophagy and protein degradation systems in myopathies and muscular dystrophies

M. Sandri, P. Bonaldo, L. Coletto, 2013, Journal of Cell Science.

Zebrafish ambra1a and ambra1b Knockdown Impairs Skeletal Muscle Development

F. Cecconi, L. Valle, V. Cianfanelli, 2014, PloS one.

Stable expression of chicken type-VI collagen α1, α2 and α3 cDNAs in murine NIH/3T3 cells

F. Bucciotti, P. Bonaldo, A. Colombatti, 1992 .

Transcriptional Activation of the α1(VI) Collagen Gene during Myoblast Differentiation Is Mediated by Multiple GA Boxes (*)

P. Bonaldo, S. Piccolo, D. Volpin, 1995, The Journal of Biological Chemistry.

The Role of Collagens in Peripheral Nerve Myelination and Function

P. Bonaldo, M. Cescon, Peiwen Chen, 2015, Molecular Neurobiology.

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

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

Altered Trabecular Bone Structure and Delayed Cartilage Degeneration in the Knees of Collagen VI Null Mice

F. Guilak, P. Bonaldo, S. Zauscher, 2012, PloS one.

Fine-tuning of ULK1 mRNA and protein levels is required for autophagy oscillation

S. Campello, F. Cecconi, C. Rodolfo, 2016, The Journal of cell biology.

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.

Cellular and molecular mechanisms of muscle atrophy

M. Sandri, P. Bonaldo, 2013, Disease Models & Mechanisms.

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.

Contributions of adipose tissue architectural and tensile properties toward defining healthy and unhealthy obesity.

P. Bonaldo, P. Scherer, Jiyoung Park, 2014, American journal of physiology. Endocrinology and metabolism.

Lack of Collagen VI Promotes Wound-Induced Hair Growth.

P. Bonaldo, M. Cescon, Peiwen Chen, 2015, The Journal of investigative dermatology.

Targeting of EMILIN-1 and EMILIN-2 to Fibrillin Microfibrils Facilitates their Incorporation into the Extracellular Matrix.

M. Paulsson, P. Bonaldo, D. Keene, 2016, The Journal of investigative dermatology.

Monoamine oxidase inhibition prevents mitochondrial dysfunction and apoptosis in myoblasts from patients with collagen VI myopathies

P. Bonaldo, P. Bernardi, F. Di Lisa, 2014, Free radical biology & medicine.

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.

Skeletal muscle, autophagy, and physical activity: the ménage à trois of metabolic regulation in health and disease

M. Sandri, A. Vainshtein, P. Bonaldo, 2013, Journal of Molecular Medicine.

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.

Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis

A. Ferlini, N. Maraldi, M. Paulsson, 2012, Matrix biology : journal of the International Society for Matrix Biology.

Physical exercise stimulates autophagy in normal skeletal muscles but is detrimental for collagen VI-deficient muscles

M. Sandri, E. Bertaggia, P. Bonaldo, 2011, Autophagy.

Type VI collagen deficiency induces osteopenia with distortion of osteoblastic cell morphology.

M. Noda, T. Hayata, T. Nakamoto, 2012, Tissue & cell.

Perinatal Lethality of Microtubule-Associated Protein 1B-Deficient Mice Expressing Alternative Isoforms of the Protein at Low Levels

E. Demandt, P. Gruss, K. Chowdhury, 2000, Molecular and Cellular Neuroscience.

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

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

Autosomal recessive Bethlem myopathy: A clinical, genetic and functional study

A. Pichiecchio, A. Padovani, A. Ferlini, 2019, Neuromuscular Disorders.

Autophagy induction rescues muscular dystrophy

M. Sandri, P. Bonaldo, L. Coletto, 2011, Autophagy.

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.

Autosomal recessive myosclerosis myopathy is a collagen VI disorder

P. Bonaldo, P. Sabatelli, L. Merlini, 2008, Neurology.

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.

S-nitrosoglutathione reductase deficiency-induced S-nitrosylation results in neuromuscular dysfunction.

C. Gargioli, F. Cecconi, G. Filomeni, 2014, Antioxidants & redox signaling.

Autophagy in Skeletal Muscle Homeostasis and in Muscular Dystrophies

P. Bonaldo, P. Grumati, 2012, Cells.

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

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

Autophagy activation in COL6 myopathic patients by a low-protein-diet pilot trial

D. Cocchi, N. Maraldi, M. Sandri, 2016, Autophagy.

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.

Mitochondrial dysfunction and defective autophagy in the pathogenesis of collagen VI muscular dystrophies.

P. Bonaldo, P. Bernardi, 2013, Cold Spring Harbor perspectives in biology.

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.

Reactivation of autophagy by spermidine ameliorates the myopathic defects of collagen VI-null mice

F. Cecconi, M. Chrisam, P. Bonaldo, 2015, Autophagy.

The epg5 knockout zebrafish line: a model to study Vici syndrome

F. Cecconi, M. Chrisam, P. Bonaldo, 2019, Autophagy.

Dysfunction of Mitochondria and Sarcoplasmic Reticulum in the Pathogenesis of Collagen VI Muscular Dystrophies

P. Bonaldo, P. Bernardi, 2008, Annals of the New York Academy of Sciences.

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

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

Autophagy in the mesh of collagen VI.

P. Bonaldo, M. Cescon, S. Castagnaro, 2020, Matrix biology : journal of the International Society for Matrix Biology.

240th ENMC workshop: The involvement of skeletal muscle stem cells in the pathology of muscular dystrophies 25–27 January 2019, Hoofddorp, The Netherlands

K. Patel, F. Muntoni, C. Bönnemann, 2019, Neuromuscular Disorders.

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..

Critical Evaluation of the Use of Cell Cultures for Inclusion in Clinical Trials of Patients Affected by Collagen VI Myopathies

P. Bonaldo, P. Bernardi, A. Angelin, 2011, Journal of cellular physiology.

Bethlem myopathy (BETHLEM) and Ullrich scleroatonic muscular dystrophy: 100th ENMC International Workshop, 23–24 November 2001, Naarden, The Netherlands

E. Bertini, B. Giusti, K. Bushby, 2002, Neuromuscular Disorders.

Tumorigenesis and Neoplastic Progression Collagen VI Ablation Retards Brain Tumor Progression Due to Deficits in Assembly of the Vascular Basal Lamina

W. You, P. Bonaldo, W. Stallcup, 2012 .

Pericyte deficiencies lead to aberrant tumor vascularizaton in the brain of the NG2 null mouse.

W. You, E. Pasquale, P. Bonaldo, 2010, Developmental biology.

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.

repeated bronchoalveolar lavage in individual mice Assessment of cellular profile and lung function with

W. Mitzner, H. Rabb, P. Bonaldo, 2015 .

Lipids and glucose homeostasis upon metabolic challenge: extracellular matrix takes the stage

P. Bonaldo, M. Cescon, 2020, The Journal of physiology.

Collagen VI ablation in zebrafish causes neuromuscular defects during developmental and adult stages.

C. Bertolucci, P. Bonaldo, P. Sabatelli, 2022, Matrix biology : journal of the International Society for Matrix Biology.

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

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

Heterogeneity of Collagen VI Microfibrils

M. Paulsson, C. Baldock, P. Bonaldo, 2016, The Journal of Biological Chemistry.

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

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

Col6a1 Null Mice as a Model to Study Skin Phenotypes in Patients with Collagen VI Related Myopathies: Expression of Classical and Novel Collagen VI Variants during Wound Healing

M. Paulsson, W. Bloch, P. Bonaldo, 2014, PloS one.

Human adipose-derived stem cell transplantation as a potential therapy for collagen VI-related congenital muscular dystrophy

P. Bonaldo, O. Igoucheva, M. Chu, 2014, Stem Cell Research & Therapy.

Antisense-induced messenger depletion corrects a COL6A2 dominant mutation in Ullrich myopathy.

A. Ferlini, E. Pegoraro, P. Bonaldo, 2012, Human gene therapy.

Differential and restricted expression of novel collagen VI chains in mouse.

M. Paulsson, P. Bonaldo, P. Sabatelli, 2011, Matrix biology : journal of the International Society for Matrix Biology.

EM.P.5.04 Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice

N. Maraldi, P. Bonaldo, P. Bernardi, 2009, Neuromuscular Disorders.

Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1

Mark R. Marten, Erinna F. Lee, Dana L. Miller, 2021, Autophagy.

An a-E-catenin gene trap mutation defines its function in preimplantation development ( cell adhesion y blastocyst y epithelial cells )

S. Butz, P. Gruss, K. Chowdhury, 1997 .

DEVELOPMENTAL AND OSTEOARTHRITIC CHANGES IN Col6a1 KNOCKOUT MICE: THE BIOMECHANICS OF COLLAGEN VI IN THE CARTILAGE PERICELLULAR MATRIX

F. Guilak, L. Alexopoulos, P. Bonaldo, 2011 .

The human type VI collagen gene. mRNA and protein variants of the alpha 3 chain generated by alternative splicing of an additional 5-end exon.

P. Bonaldo, A. Colombatti, R. Doliana, 1992, The Journal of biological chemistry.

Type VI Collagen Regulates Pericellular Matrix Properties, Chondrocyte Swelling, and Mechanotransduction in Mouse Articular Cartilage

F. Guilak, P. Bonaldo, H. Leddy, 2015, Arthritis & rheumatology.

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.

Perturbations in cell signaling elicit early cardiac defects in mucopolysaccharidosis type II

R. Tomanin, A. Urbani, M. Filocamo, 2017, Human molecular genetics.

Autophagy in health and disease

F. Cecconi, P. Bonaldo, 2020, Progress in Molecular Biology and Translational Science.

Secretion and Matrix Assembly of Recombinant Type VI Collagen (*)

P. Bonaldo, A. Colombatti, M. Mucignat, 1995, The Journal of Biological Chemistry.

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.

Altered expression of the MCSP/NG2 chondroitin sulfate proteoglycan in collagen VI deficiency

E. Bertini, B. Giusti, R. Carrozzo, 2005, Molecular and Cellular Neuroscience.

Congenital muscular dystrophy-associated inflammatory chemokines provide axes for effective recruitment of therapeutic adult stem cell into muscles

P. Bonaldo, O. Igoucheva, L. Merlini, 2020, Stem cell research & therapy.

Absence of Type VI Collagen Paradoxically Improves Cardiac Function, Structure, and Remodeling After Myocardial Infarction

D. Weihrauch, P. Bonaldo, W. Chilian, 2012, Circulation research.

Efficient Expression of Chicken α(VI) Collagen Chain in Transiently Transfected Mammalian Cells

P. Bonaldo, A. Colombatti, M. Mucignat, 1990 .

Identification of a deep intronic mutation in the COL6A2 gene by a novel custom oligonucleotide CGH array designed to explore allelic and genetic heterogeneity in collagen VI-related myopathies

E. Bertini, E. Mercuri, A. Ferlini, 2010, BMC Medical Genetics.

Autosomal recessive Bethlem myopathy

E. Mercuri, A. Ferlini, S. Messina, 2009, Neurology.

On the pathogenesis of collagen VI muscular dystrophies--comment on article of Hicks et al.

N. Maraldi, P. Bonaldo, P. Bernardi, 2009, Brain : a journal of neurology.

The carboxyl terminus of the chicken alpha 3 chain of collagen VI is a unique mosaic structure with glycoprotein Ib-like, fibronectin type III, and Kunitz modules.

P. Bonaldo, A. Colombatti, 1989, The Journal of biological chemistry.

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.

The notochord: structure and functions

P. Bonaldo, Diana Corallo, Valeria Trapani, 2015, Cellular and Molecular Life Sciences.

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.

Role of macrophages in Wallerian degeneration and axonal regeneration after peripheral nerve injury

P. Bonaldo, X. Piao, Peiwen Chen, 2015, Acta Neuropathologica.

Metabolic Dysregulation and Adipose Tissue Fibrosis: Role of Collagen VI

Philipp E. Scherer, Puneeth Iyengar, Nicola Abate, 2008, Molecular and Cellular Biology.

Emilin1 Links TGF-β Maturation to Blood Pressure Homeostasis

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

Dysfunctional tendon collagen fibrillogenesis in collagen VI null mice.

L. Soslowsky, P. Bonaldo, D. Birk, 2011, Matrix biology : journal of the International Society for Matrix Biology.

Structural and functional features of the alpha 3 chain indicate a bridging role for chicken collagen VI in connective tissues.

F. Bucciotti, P. Bonaldo, A. Colombatti, 1990, Biochemistry.

Type A modules: interacting domains found in several non-fibrillar collagens and in other extracellular matrix proteins.

P. Bonaldo, A. Colombatti, R. Doliana, 1993, Matrix.

Role of macrophage polarization in tumor angiogenesis and vessel normalization: implications for new anticancer therapies.

P. Bonaldo, Peiwen Chen, 2013, International review of cell and molecular biology.

Physical mapping of mouse collagen genes on Chromosome 10 by high-resolution FISH

A. Palotie, E. Vuorio, I. Eerola, 2001, Mammalian Genome.

Transcription Factor EB Controls Metabolic Flexibility during Exercise

A. Ballabio, M. Zeviani, P. Saha, 2017, Cell metabolism.

Identification and characterization of novel collagen VI non‐canonical splicing mutations causing ullrich congenital muscular dystrophy

E. Bertini, M. Pane, E. Mercuri, 2009, Human mutation.

An alpha-E-catenin gene trap mutation defines its function in preimplantation development.

S. Butz, P. Gruss, K. Chowdhury, 1997, Proceedings of the National Academy of Sciences of the United States of America.

Type VI collagen expression is upregulated in the early events of chondrocyte differentiation.

R. Cancedda, R. Quarto, P. Bonaldo, 1993, Development.

EMILIN2 down‐modulates the Wnt signalling pathway and suppresses breast cancer cell growth and migration

P. Bonaldo, A. Colombatti, S. Marastoni, 2014, The Journal of pathology.

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.

Autophagy is Impaired in the Tibialis Anterior of Dystrophin Null Mice

Pietro Spitali, M. Hiller, P. Spitali, 2013, PLoS currents.

CMG2/ANTXR2 regulates extracellular collagen VI which accumulates in hyaline fibromatosis syndrome

L. Abrami, F. G. van der Goot, P. Bonaldo, 2017, Nature Communications.

Collagen VI regulates satellite cell self-renewal and muscle regeneration

Marco Quarta, Giulio Cossu, Giovanni Vozzi, 2013, Nature Communications.

Three Novel Collagen VI Chains with High Homology to the α3 Chain*

M. Paulsson, P. Bonaldo, M. Koch, 2008, Journal of Biological Chemistry.

Murine alpha 1(VI) collagen chain. Complete amino acid sequence and identification of the gene promoter region.

P. Bonaldo, S. Piccolo, D. Volpin, 1993, Matrix.

Collagen VI in healthy and diseased nervous system

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

Multiple forms of chicken alpha 3(VI) collagen chain generated by alternative splicing in type A repeated domains

P. Bonaldo, A. Colombatti, R. Doliana, 1990, The Journal of cell biology.

Monoclonal antibodies for the different chains of chick type VI collagen.

P. Bonaldo, A. Colombatti, M. Mucignat, 1988, Collagen and related research.

Biosynthesis of chick type VI collagen. I. Intracellular assembly and molecular structure.

P. Bonaldo, A. Colombatti, D. Volpin, 1987, The Journal of biological chemistry.

Extracellular matrix: A dynamic microenvironment for stem cell niche

P. Bonaldo, F. Gattazzo, Paolo Bonaldo, 2014, Biochimica et biophysica acta.

Alpha 1 chain of chick type VI collagen. The complete cDNA sequence reveals a hybrid molecule made of one short collagen and three von Willebrand factor type A-like domains.

F. Bucciotti, P. Bonaldo, A. Colombatti, 1989 .

Spatio-temporal expression and distribution of collagen VI during zebrafish development

P. Bonaldo, F. Ruggiero, R. Wagener, 2019, Scientific Reports.

The α1 Chain of Chick Type VI Collagen Is a Hybrid Molecule Made of One Short Collagen and Three von Willebrand Factor Type A‐like Domains a

F. Bucciotti, P. Bonaldo, A. Colombatti, 1990, The Journal of biological chemistry.

The elastin associated glycoprotein gp115. Synthesis and secretion by chick cells in culture.

P. Bonaldo, A. Colombatti, D. Volpin, 1988, The Journal of biological chemistry.

EMILIN-1 Deficiency Induces Elastogenesis and Vascular Cell Defects

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

Collagen VI-NG2 axis in human tendon fibroblasts under conditions mimicking injury response.

F. Traina, N. Maraldi, P. Bonaldo, 2016, Matrix biology : journal of the International Society for Matrix Biology.

Annexin A2 mediates secretion of collagen VI, pulmonary elasticity and apoptosis of bronchial epithelial cells

P. Bonaldo, S. Worgall, K. Hajjar, 2014, Journal of Cell Science.

Isolation of cDNA clones corresponding to the Mr = 150,000 subunit of chick type VI collagen.

F. Bucciotti, P. Bonaldo, A. Colombatti, 1987, Biochemical and biophysical research communications.

Cyclosporine A in Ullrich Congenital Muscular Dystrophy: Long-Term Results

E. Bertini, A. Ferlini, N. Maraldi, 2011, Oxidative medicine and cellular longevity.

212th ENMC International Workshop:: Animal models of congenital muscular dystrophies, Naarden, The Netherlands, 29–31 May 2015

Susan C. Brown, M. Girgenrath, C. Bönnemann, 2016, Neuromuscular Disorders.

Lack of collagen VI promotes neurodegeneration by impairing autophagy and inducing apoptosis during aging

P. Bonaldo, M. Cescon, Peiwen Chen, 2016, Aging.

Cardiac glycoside ouabain induces autophagic cell death in non-small cell lung cancer cells via a JNK-dependent decrease of Bcl-2.

P. Bonaldo, P. Grumati, A. Trenti, 2014, Biochemical pharmacology.

First steps to define murine amniotic fluid stem cell microenvironment

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

Developmental and osteoarthritic changes in Col6a1-knockout mice: biomechanics of type VI collagen in the cartilage pericellular matrix.

F. Guilak, L. Alexopoulos, P. Bonaldo, 2009, Arthritis and rheumatism.

The Role of Collagens in Peripheral Nerve Myelination and Function

P. Bonaldo, M. Cescon, Peiwen Chen, 2015, Molecular Neurobiology.

Evidence for the stochastic integration of gene trap vectors into the mouse germline.

P. Gruss, K. Chowdhury, P. Bonaldo, 1997, Nucleic acids research.

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.

EMI, a novel cysteine‐rich domain of EMILINs and other extracellular proteins, interacts with the gC1q domains and participates in multimerization

P. Bonaldo, A. Colombatti, R. Doliana, 2000, FEBS letters.

Collagen VI regulates peripheral nerve regeneration by modulating macrophage recruitment and polarization

L. Nobbio, P. Bonaldo, M. Cescon, 2014, Acta Neuropathologica.

Editorial: Pathophysiological Mechanisms of Sarcopenia in Aging and in Muscular Dystrophy: A Translational Approach

P. Bonaldo, E. Marzetti, L. Merlini, 2015, Front. Aging Neurosci..

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.

Bicalutamide and Trehalose Ameliorate Spinal and Bulbar Muscular Atrophy Pathology in Mice

A. Vercelli, M. Pennuto, N. Mitro, 2023, Neurotherapeutics.

Glycolytic-to-oxidative fiber-type switch and mTOR signaling activation are early-onset features of SBMA muscle modified by high-fat diet

M. Pellegrini, F. Sambataro, M. Pennuto, 2016, Acta Neuropathologica.

Gene trap expression and mutational analysis for genes involved in the development of the mammalian nervous system

P. Gruss, K. Chowdhury, P. Bonaldo, 1998, Developmental dynamics : an official publication of the American Association of Anatomists.

Aggresome–Autophagy Involvement in a Sarcopenic Patient with Rigid Spine Syndrome and a p.C150R Mutation in FHL1 Gene

N. Maraldi, M. Sandri, M. Chrisam, 2014, Front. Aging Neurosci..

Collagen VI Deficiency Results in Structural Abnormalities in the Mouse Lung.

S. Bhattacharya, T. Mariani, P. Bonaldo, 2019, The American journal of pathology.

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

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

Zebrafish ambra1b knockout reveals a novel role for Ambra1 in primordial germ cells survival, sex differentiation and reproduction

F. Cecconi, F. Nazio, P. Bonaldo, 2023, Biological Research.

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.

Autophagy-mediated regulation of macrophages and its applications for cancer

P. Bonaldo, M. Cescon, Peiwen Chen, 2014, Autophagy.

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.

Skeletal muscle, autophagy, and physical activity: the ménage à trois of metabolic regulation in health and disease

M. Sandri, A. Vainshtein, P. Bonaldo, 2014, Journal of Molecular Medicine.

Collagen VI is required for the structural and functional integrity of the neuromuscular junction

E. Pegoraro, P. Bonaldo, S. Hashemolhosseini, 2018, Acta Neuropathologica.

Characterization of a rare case of Ullrich congenital muscular dystrophy due to truncating mutations within the COL6A1 gene C-Terminal domain: a case report

E. Bertini, A. D’Amico, S. Petrini, 2013, BMC Medical Genetics.

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.

The notochord: structure and functions

P. Bonaldo, Diana Corallo, Valeria Trapani, 2015, Cellular and Molecular Life Sciences.

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.

Ultrastructural defects of collagen VI filaments in an Ullrich syndrome patient with loss of the α3(VI) N10‐N7 domains

N. Maraldi, C. Capanni, P. Bonaldo, 2006, Journal of cellular physiology.

Structure, Chromosomal Localization, and Promoter Analysis of the Human Elastin MicrofibrilInterfase Located proteIN (EMILIN) Gene*

F. Bucciotti, P. Bonaldo, A. Colombatti, 2000, The Journal of Biological Chemistry.

Erratum to: Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (Autophagy, 12, 1, 1-222, 10.1080/15548627.2015.1100356

Sara W. Bird, Erinna F. Lee, Michael D. Kim, 2016 .

Collagen VI protects neurons against Aβ toxicity

L. Mucke, T. Wyss-Coray, I. Tesseur, 2009, Nature Neuroscience.

Editorial :Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition). DOI: 10.1080/15548627.2015.1100356;WOS:000373595400001; 2-s2.0-85013763791&;PMID: 26799652

Lee, Duchen, Izzo, 2016 .

Spatio-temporal expression and distribution of collagen VI during zebrafish development

P. Bonaldo, F. Ruggiero, R. Wagener, 2019, Scientific Reports.

P5 Detection of circulating miRNAs in serum in a mouse model of Collagen VI Deficiency

F. Muntoni, E. Pegoraro, P. Bonaldo, 2014, Neuromuscular Disorders.

Expression of the collagen VI α5 and α6 chains in normal human skin and in skin of patients with collagen VI-related myopathies.

M. Paulsson, P. Bonaldo, A. Zamparelli, 2011, The Journal of investigative dermatology.

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.

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.

WITHDRAWN: Autosomal recessive Bethlem myopathy: A clinical, genetic and functional study

A. Pichiecchio, A. Padovani, A. Ferlini, 2019, Neuromuscular Disorders.

Collagen VI sustains cell stemness and chemotherapy resistance in glioblastoma

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

Cellular and molecular mechanisms of muscle atrophy

M. Sandri, P. Bonaldo, 2013, Disease Models & Mechanisms.

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

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

Mitochondrial dysfunction and defective autophagy in the pathogenesis of collagen VI muscular dystrophies.

P. Bonaldo, P. Bernardi, 2013, Cold Spring Harbor perspectives in biology.

Autosomal recessive myosclerosis myopathy is a collagen VI disorder

P. Bonaldo, P. Sabatelli, P. Grumati, 2008, Neurology.

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.

Metabolic Dysregulation and Adipose Tissue Fibrosis: Role of Collagen VI

P. Iyengar, N. Abate, M. Chandalia, 2008, Molecular and Cellular Biology.

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

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

Guidelines for the use and interpretation of assays for monitoring autophage

T. P. Neufeld, Thomas J. Begley, H. Coller, 2012 .

Contributions of adipose tissue architectural and tensile properties toward defining healthy and unhealthy obesity.

P. Bonaldo, P. Scherer, Jiyoung Park, 2014, American journal of physiology. Endocrinology and metabolism.

Detecting Collagen VI in Bethlem Myopathy

P. Bonaldo, P. Sabatelli, L. Merlini, 2015, The Journal of Biological Chemistry.

Integrative Physiology Absence of Type Vi Collagen Paradoxically Improves Cardiac Function, Structure, and Remodeling after Myocardial Infarction Short Communication Methods and Results: Wild-type and Col6a1 Methods Animal Model Surgical Induction of Mi Histological Assessment of Apoptosis, Fibrosis

P. Bonaldo, W. Chilian, C. Hodnichak, 2022 .

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

Collagen VI is required for the structural and functional integrity of the neuromuscular junction

E. Pegoraro, P. Bonaldo, S. Hashemolhosseini, 2018, Acta Neuropathologica.

Sustained oral spermidine supplementation rescues functional and structural defects in COL6-deficient myopathic mice

P. Bonaldo, P. Sabatelli, M. Cescon, 2023, Autophagy.

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.

Type VI collagen expression is upregulated in the early events of chondrocyte differentiation

R. Quarto, P. Bonaldo, B. Dozin, 1996 .

Type VI collagen expression is upregulated in the early events of chondrocyte differentiation

R. Quarto, P. Bonaldo, B. Dozin, 1996 .

Type VI collagen expression is upregulated in the early events of chondrocyte differentiation.

R. Quarto, P. Bonaldo, B. Dozin, 1993, Development.