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Histone deacetylases in Duchenne muscular dystrophy: a role in the mechanism of disease and a target for inhibition
by
Albamonte, Emilio
, Masson, Riccardo
, Panicucci, Chiara
, Ricci, Federica
, Bertoldi, Mariarita
, Nigro, Vincenzo
, Bello, Luca
, Pane, Marika
, D’amico, Adele
, Sframeli, Maria
in
Adipose tissue
/ Animals
/ Biomedical and Life Sciences
/ Biomedicine
/ Biosynthesis
/ Chromatin
/ Development and progression
/ Disease
/ Diseases
/ Duchenne muscular dystrophy
/ Duchenne's muscular dystrophy
/ Dystrophin
/ Dystrophin - metabolism
/ Enzymes
/ Epigenesis, Genetic
/ Epigenetic inheritance
/ Epigenetics
/ Gene expression
/ Gene Function
/ Genes
/ Genotype & phenotype
/ Histone deacetylase
/ Histone Deacetylase Inhibitors - pharmacology
/ Histone Deacetylase Inhibitors - therapeutic use
/ Histone Deacetylases - genetics
/ Histone Deacetylases - metabolism
/ Histones
/ Homeostasis
/ Human Genetics
/ Humans
/ Hyperactivity
/ Immune response
/ Immune system
/ Mechanical properties
/ MicroRNAs
/ Muscle, Skeletal
/ Muscular dystrophy
/ Muscular Dystrophy, Duchenne - drug therapy
/ Muscular Dystrophy, Duchenne - enzymology
/ Muscular Dystrophy, Duchenne - genetics
/ Musculoskeletal system
/ Nitric oxide
/ Physiological aspects
/ Physiology
/ Proteins
/ Repair & maintenance
/ Review
/ Signal Transduction
/ Transcription factors
/ Utrophin
2025
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Histone deacetylases in Duchenne muscular dystrophy: a role in the mechanism of disease and a target for inhibition
by
Albamonte, Emilio
, Masson, Riccardo
, Panicucci, Chiara
, Ricci, Federica
, Bertoldi, Mariarita
, Nigro, Vincenzo
, Bello, Luca
, Pane, Marika
, D’amico, Adele
, Sframeli, Maria
in
Adipose tissue
/ Animals
/ Biomedical and Life Sciences
/ Biomedicine
/ Biosynthesis
/ Chromatin
/ Development and progression
/ Disease
/ Diseases
/ Duchenne muscular dystrophy
/ Duchenne's muscular dystrophy
/ Dystrophin
/ Dystrophin - metabolism
/ Enzymes
/ Epigenesis, Genetic
/ Epigenetic inheritance
/ Epigenetics
/ Gene expression
/ Gene Function
/ Genes
/ Genotype & phenotype
/ Histone deacetylase
/ Histone Deacetylase Inhibitors - pharmacology
/ Histone Deacetylase Inhibitors - therapeutic use
/ Histone Deacetylases - genetics
/ Histone Deacetylases - metabolism
/ Histones
/ Homeostasis
/ Human Genetics
/ Humans
/ Hyperactivity
/ Immune response
/ Immune system
/ Mechanical properties
/ MicroRNAs
/ Muscle, Skeletal
/ Muscular dystrophy
/ Muscular Dystrophy, Duchenne - drug therapy
/ Muscular Dystrophy, Duchenne - enzymology
/ Muscular Dystrophy, Duchenne - genetics
/ Musculoskeletal system
/ Nitric oxide
/ Physiological aspects
/ Physiology
/ Proteins
/ Repair & maintenance
/ Review
/ Signal Transduction
/ Transcription factors
/ Utrophin
2025
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Histone deacetylases in Duchenne muscular dystrophy: a role in the mechanism of disease and a target for inhibition
by
Albamonte, Emilio
, Masson, Riccardo
, Panicucci, Chiara
, Ricci, Federica
, Bertoldi, Mariarita
, Nigro, Vincenzo
, Bello, Luca
, Pane, Marika
, D’amico, Adele
, Sframeli, Maria
in
Adipose tissue
/ Animals
/ Biomedical and Life Sciences
/ Biomedicine
/ Biosynthesis
/ Chromatin
/ Development and progression
/ Disease
/ Diseases
/ Duchenne muscular dystrophy
/ Duchenne's muscular dystrophy
/ Dystrophin
/ Dystrophin - metabolism
/ Enzymes
/ Epigenesis, Genetic
/ Epigenetic inheritance
/ Epigenetics
/ Gene expression
/ Gene Function
/ Genes
/ Genotype & phenotype
/ Histone deacetylase
/ Histone Deacetylase Inhibitors - pharmacology
/ Histone Deacetylase Inhibitors - therapeutic use
/ Histone Deacetylases - genetics
/ Histone Deacetylases - metabolism
/ Histones
/ Homeostasis
/ Human Genetics
/ Humans
/ Hyperactivity
/ Immune response
/ Immune system
/ Mechanical properties
/ MicroRNAs
/ Muscle, Skeletal
/ Muscular dystrophy
/ Muscular Dystrophy, Duchenne - drug therapy
/ Muscular Dystrophy, Duchenne - enzymology
/ Muscular Dystrophy, Duchenne - genetics
/ Musculoskeletal system
/ Nitric oxide
/ Physiological aspects
/ Physiology
/ Proteins
/ Repair & maintenance
/ Review
/ Signal Transduction
/ Transcription factors
/ Utrophin
2025
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Histone deacetylases in Duchenne muscular dystrophy: a role in the mechanism of disease and a target for inhibition
Journal Article
Histone deacetylases in Duchenne muscular dystrophy: a role in the mechanism of disease and a target for inhibition
2025
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Overview
Aberrant activity of histone deacetylases (HDACs) is a pathological phenomenon in several diseases, including Duchenne muscular dystrophy (DMD). In DMD, the upregulation of HDACs is driven by the disassembly of the dystrophin-associated protein complex (DAPC), which, under normal physiological conditions, provides mechanical stability to muscle fibres and acts as a signalling hub anchoring signalling proteins and molecules to their functional sites. In dystrophic muscle, DAPC disassembly causes delocalisation of signalling proteins and, therefore, disrupts signalling pathways. Displacement of epigenetic signalling molecules leads to the uncontrolled activity of HDACs and excessive removal of acetyl groups from histone proteins. Consequently, chromatin becomes tightly bound, preventing the expression of genes involved in muscle homeostasis. The pathological consequences of increased HDAC activity extend beyond muscle fibres, affecting several cell types, translating into a chronically activated immune system, promoting fibrotic and adipose tissue formation and impairing muscle regeneration. Here, we review the current evidence implicating HDACs as a key driver in DMD disease development and progression. We describe the mechanism of HDAC overactivity and the downstream consequences that contribute to the pathogenesis of the disease by disrupting muscle repair and regeneration. Finally, we highlight HDACs as targets for inhibition, offering a novel therapeutic strategy to counteract the multiple pathological events in DMD.
Graphical abstract
Publisher
BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
Subject
/ Animals
/ Biomedical and Life Sciences
/ Disease
/ Diseases
/ Duchenne's muscular dystrophy
/ Enzymes
/ Genes
/ Histone Deacetylase Inhibitors - pharmacology
/ Histone Deacetylase Inhibitors - therapeutic use
/ Histone Deacetylases - genetics
/ Histone Deacetylases - metabolism
/ Histones
/ Humans
/ Muscular Dystrophy, Duchenne - drug therapy
/ Muscular Dystrophy, Duchenne - enzymology
/ Muscular Dystrophy, Duchenne - genetics
/ Proteins
/ Review
/ Utrophin
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