Search results for: Pier Lorenzo Puri

Items from 1 to 20 out of 32 results

article

Shaping Gene Expression by Landscaping Chromatin Architecture: Lessons from a Master

Vittorio Sartorelli, Pier Lorenzo Puri

Molecular Cell > 2018 > 71 > 3 > 375-388

Since its discovery as a skeletal muscle-specific transcription factor able to reprogram somatic cells into differentiated myofibers, MyoD has provided an instructive model to understand how transcription factors regulate gene expression. Reciprocally, studies of other transcriptional regulators have provided testable hypotheses to further understand how MyoD activates transcription. Using MyoD as...

article

Reversal of Defective Mitochondrial Biogenesis in Limb-Girdle Muscular Dystrophy 2D by Independent Modulation of Histone and PGC-1α Acetylation

Sarah Pambianco, Matteo Giovarelli, Cristiana Perrotta, Silvia Zecchini, more

Cell Reports > 2016 > 17 > 11 > 3010-3023

Mitochondrial dysfunction occurs in many muscle degenerative disorders. Here, we demonstrate that mitochondrial biogenesis was impaired in limb-girdle muscular dystrophy (LGMD) 2D patients and mice and was associated with impaired OxPhos capacity. Two distinct approaches that modulated histones or peroxisome proliferator-activated receptor-gamma coactivator 1 α (PGC-1α) acetylation exerted equivalent...

article

Histological effects of givinostat in boys with Duchenne muscular dystrophy

Paolo Bettica, Stefania Petrini, Valentina D'Oria, Adele D'Amico, more

Neuromuscular Disorders > 2016 > 26 > 10 > 643-649

Duchenne Muscular Dystrophy (DMD) is caused by mutations in the dystrophin gene leading to dystrophin deficiency, muscle fiber degeneration and progressive fibrotic replacement of muscles. Givinostat, a histone deacetylase (HDAC) inhibitor, significantly reduced fibrosis and promoted compensatory muscle regeneration in mdx mice. This study was conducted to evaluate whether the beneficial histological...

article

Could we also be regenerative superheroes, like salamanders?

Alessandra Dall'Agnese, Pier Lorenzo Puri

BioEssays > 38 > 9 > 917 - 926

Development of methods to reawaken the semi‐dormant regenerative potential that lies within adult human tissues would hold promise for the restoration of diseased or damaged organs and tissues. While most of the regeneration potential is suppressed in many vertebrates, including humans, during adult life, urodele amphibians (salamanders) retain their regenerative ability throughout adulthood. Studies...

article

SWI/SNF-directed stem cell lineage specification: dynamic composition regulates specific stages of skeletal myogenesis

Paula Coutinho Toto, Pier Lorenzo Puri, Sonia Albini

Cellular and Molecular Life Sciences > 2016 > 73 > 20 > 3887-3896

SWI/SNF chromatin-remodeling complexes are key regulators of the epigenetic modifications that determine whether stem cells maintain pluripotency or commit toward specific lineages through development and during postnatal life. Dynamic combinatorial assembly of multiple variants of SWI/SNF subunits is emerging as the major determinant of the functional versatility of SWI/SNF. Here, we summarize the...

article

Regulation of Muscle Satellite Cell Function in Tissue Homeostasis and Aging

Alessandra Sacco, Pier Lorenzo Puri

Cell Stem Cell > 2015 > 16 > 6 > 585-587

Age-related muscle decline is associated with functional impairment of satellite cells (SCs). Conflicting data suggest dysregulation of cell-extrinsic or -intrinsic factors can independently contribute to such impairment. Here, we emphasize the importance of identifying nodes that integrate these factors into feed-forward circuits, which could provide targets for therapeutic intervention.

article

Redox or Death: Checking on Fetal Myogenesis

Lucia Latella, Pier Lorenzo Puri

Developmental Cell > 2014 > 29 > 4 > 373-374

Reporting in this issue of Developmental Cell, L’honoré et al. (2014) show that a network composed of Pitx2/Pitx3 and downstream antioxidant enzymes protects differentiating skeletal muscle from excessive reactive oxygen species production during fetal myogenesis. Genetic deficiency of Pitx2/Pitx3 results in irreversible oxidative DNA damage and apoptosis, impairing skeletal muscle development.

article

Nitric Oxide and Histone Acetylation—Shaping Craniofacial Development

Libera Berghella, Pier Lorenzo Puri

Chemistry & Biology > 2014 > 21 > 5 > 565-566

Previous work linked nitric oxide (NO) signaling to histone deacetelyases (HDACs) in the control of tissue homeostasis and suggested that deregulation of this signaling contributes to human diseases. In the previous issue of Chemistry & Biology, Kong and colleagues showed that coordinated NO signaling and histone acetylation are required for proper cranial neural crest development and craniofacial...

article

Epigenetic control of skeletal muscle regeneration

Lorenzo Giordani, Pier Lorenzo Puri

The FEBS Journal > 280 > 17 > 4014 - 4025

During embryonic development, pluripotent cells are genetically committed to specific lineages by the expression of cell‐type‐specific transcriptional activators that direct the formation of specialized tissues and organs in response to developmental cues. Chromatin‐modifying proteins are emerging as essential components of the epigenetic machinery, which establishes the nuclear landscape that ultimately...

article

Epigenetic Reprogramming of Human Embryonic Stem Cells into Skeletal Muscle Cells and Generation of Contractile Myospheres

Sonia Albini, Paula Coutinho, Barbora Malecova, Lorenzo Giordani, more

Cell Reports > 2013 > 3 > 3 > 661-670

Direct generation of a homogeneous population of skeletal myoblasts from human embryonic stem cells (hESCs) and formation of three-dimensional contractile structures for disease modeling in vitro are current challenges in regenerative medicine. Previous studies reported on the generation of myoblasts from ESC-derived embryoid bodies (EB), but not from undifferentiated ESCs, indicating the requirement...

article

A novel AMPK-dependent FoxO3A-SIRT3 intramitochondrial complex sensing glucose levels

Alessia Peserico, Fulvio Chiacchiera, Valentina Grossi, Antonio Matrone, more

Cellular and Molecular Life Sciences > 2013 > 70 > 11 > 2015-2029

Reduction of nutrient intake without malnutrition positively influences lifespan and healthspan from yeast to mice and exerts some beneficial effects also in humans. The AMPK-FoxO axis is one of the evolutionarily conserved nutrient-sensing pathways, and the FOXO3A locus is associated with human longevity. Interestingly, FoxO3A has been reported to be also a mitochondrial protein in mammalian cells...

article

Preclinical Studies in the mdx Mouse Model of Duchenne Muscular Dystrophy with the Histone Deacetylase Inhibitor Givinostat

Silvia Consalvi, Chiara Mozzetta, Paolo Bettica, Massimiliano Germani, more

Molecular Medicine > 2013 > 19 > 1 > 79-87

Previous work has established the existence of dystrophin-nitric oxide (NO) signaling to histone deacetylases (HDACs) that is deregulated in dystrophic muscles. As such, pharmacological interventions that target HDACs (that is, HDAC inhibitors) are of potential therapeutic interest for the treatment of muscular dystrophies. In this study, we explored the effectiveness of long-term treatment with different...

article

SIRT1 signaling as potential modulator of skeletal muscle diseases

Joanne Tonkin, Francesc Villarroya, Pier Lorenzo Puri, Manlio Vinciguerra

Current Opinion in Pharmacology > 2012 > 12 > 3 > 372-376

Skeletal muscle diseases heavily impair the strength and the movement of patients. Muscles loose their adaptive capacity, undergoing atrophy or wasting, due to a number of pathological insults. Metabolic changes, such as those occurring during aging, contribute to the progressive reduction of myofiber size and decline of skeletal muscle performance that is typically observed in the elderly. The nicotinamide...

article

Differentiation of human rhabdomyosarcoma RD cells is regulated by reciprocal, functional interactions between myostatin, p38 and extracellular regulated kinase signalling pathways

Stefania Rossi, Elena Stoppani, Pier Lorenzo Puri, Alessandro Fanzani

European Journal of Cancer > 2011 > 47 > 7 > 1095-1105

Rhabdomyosarcoma (RMS) includes heterogeneous tumours of mesenchymal derivation which are genetically committed to the myogenic lineage, but fail to complete terminal differentiation. Previous works have reported on deregulated myostatin, p38 and extracellular regulated kinase (ERK) signalling in RMS cell lines; however, the functional link between these pathways and their relative contribution to...

article

Phosphoryl-EZH-ion

Giuseppina Caretti, Daniela Palacios, Vittorio Sartorelli, Pier Lorenzo Puri

Cell Stem Cell > 2011 > 8 > 3 > 262-265

Polycomb group (PcG) proteins regulate gene expression in embryonic and adult stem cells, but the mechanisms responsible for PcG gene targeting and regulation remain largely unknown. Recent evidence shows that EZH2, the enzymatic subunit of Polycomb Repressive Complex 2 (PRC2), is a nuclear phosphoprotein linking cell-cycle-intrinsic or extracellular signals to specific epigenetic signatures.

article

Histone Deacetylase Inhibitors in the Treatment of Muscular Dystrophies: Epigenetic Drugs for Genetic Diseases

Silvia Consalvi, Valentina Saccone, Lorenzo Giordani, Giulia Minetti, more

Molecular Medicine > 2011 > 17 > 5-6 > 457-465

Histone deacetylases inhibitors (HDACi) include a growing number of drugs that share the ability to inhibit the enzymatic activity of some or all the HDACs. Experimental and preclinical evidence indicates that these epigenetic drugs not only can be effective in the treatment of malignancies, inflammatory diseases and degenerative disorders, but also in the treatment of genetic diseases, such as muscular...

article

SWI/SNF complexes, chromatin remodeling and skeletal myogenesis: It's time to exchange!

Sonia Albini, Pier Lorenzo Puri

Experimental Cell Research > 2010 > 316 > 18 > 3073-3080

Skeletal muscle differentiation relies on the coordinated activation and repression of specific subsets of genes. This reflects extensive changes in chromatin architecture, composition of chromatin-associated complexes and histone modifications at the promoter/enhancer elements of skeletal muscle genes. An early, key event in the activation of muscle-specific gene transcription is the disruption of...

article

TNF/p38α/Polycomb Signaling to Pax7 Locus in Satellite Cells Links Inflammation to the Epigenetic Control of Muscle Regeneration

Daniela Palacios, Chiara Mozzetta, Silvia Consalvi, Giuseppina Caretti, more

Cell Stem Cell > 2010 > 7 > 4 > 455-469

How regeneration cues are converted into the epigenetic information that controls gene expression in adult stem cells is currently unknown. We identified an inflammation-activated signaling in muscle stem (satellite) cells, by which the polycomb repressive complex 2 (PRC2) represses Pax7 expression during muscle regeneration. TNF-activated p38α kinase promotes the interaction between YY1 and PRC2,...

article

HDACs and sirtuins: Targets for new pharmacological interventions in human diseases

Pier Lorenzo Puri, Vittorio Sartorelli

Pharmacological Research > 2010 > 62 > 1 > 1-2

article

Switch NFix Developmental Myogenesis

Daniela Palacios, Pier Lorenzo Puri

Developmental Cell > 2010 > 18 > 3 > 340-341

During development, skeletal muscles adapt to stage-specific functional and metabolic challenges by switching the expression of specific subset of genes. The mechanism that governs these changes is still enigmatic. In a recent issue of Cell, Messina and coworkers shed light on this issue through the identification of a transcription factor—NFix—that coordinates the switch in gene expression at the...

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INFONA - science communication portal

Search results for: Pier Lorenzo Puri

Shaping Gene Expression by Landscaping Chromatin Architecture: Lessons from a Master

Reversal of Defective Mitochondrial Biogenesis in Limb-Girdle Muscular Dystrophy 2D by Independent Modulation of Histone and PGC-1α Acetylation

Histological effects of givinostat in boys with Duchenne muscular dystrophy

Could we also be regenerative superheroes, like salamanders?

SWI/SNF-directed stem cell lineage specification: dynamic composition regulates specific stages of skeletal myogenesis

Regulation of Muscle Satellite Cell Function in Tissue Homeostasis and Aging

Redox or Death: Checking on Fetal Myogenesis

Nitric Oxide and Histone Acetylation—Shaping Craniofacial Development

Epigenetic control of skeletal muscle regeneration

Epigenetic Reprogramming of Human Embryonic Stem Cells into Skeletal Muscle Cells and Generation of Contractile Myospheres

A novel AMPK-dependent FoxO3A-SIRT3 intramitochondrial complex sensing glucose levels

Preclinical Studies in the mdx Mouse Model of Duchenne Muscular Dystrophy with the Histone Deacetylase Inhibitor Givinostat

SIRT1 signaling as potential modulator of skeletal muscle diseases

Differentiation of human rhabdomyosarcoma RD cells is regulated by reciprocal, functional interactions between myostatin, p38 and extracellular regulated kinase signalling pathways

Phosphoryl-EZH-ion

Histone Deacetylase Inhibitors in the Treatment of Muscular Dystrophies: Epigenetic Drugs for Genetic Diseases

SWI/SNF complexes, chromatin remodeling and skeletal myogenesis: It's time to exchange!

TNF/p38α/Polycomb Signaling to Pax7 Locus in Satellite Cells Links Inflammation to the Epigenetic Control of Muscle Regeneration

HDACs and sirtuins: Targets for new pharmacological interventions in human diseases

Switch NFix Developmental Myogenesis

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