A5088308430- Muscle Physiology and Disorders
- Ion channel regulation and function
- Ion Channels and Receptors
- Exercise and Physiological Responses
- Adipose Tissue and Metabolism
- Neurobiology and Insect Physiology Research
- Thermoregulation and physiological responses
- Mitochondrial Function and Pathology
- Cardiomyopathy and Myosin Studies
- Genetic Neurodegenerative Diseases
- Muscle activation and electromyography studies
- Postharvest Quality and Shelf Life Management
- Cardiovascular Effects of Exercise
- Endoplasmic Reticulum Stress and Disease
- Climate Change and Health Impacts
- Galectins and Cancer Biology
- Cardiac electrophysiology and arrhythmias
- Neurogenetic and Muscular Disorders Research
- Autophagy in Disease and Therapy
- Erythrocyte Function and Pathophysiology
- Amyotrophic Lateral Sclerosis Research
- Genetics and Neurodevelopmental Disorders
- Muscle metabolism and nutrition
- Nutrition and Health in Aging
- Histone Deacetylase Inhibitors Research
University of Chieti-Pescara
2016-2025
Universidade Estadual de Londrina
2022
Meyer Children's Hospital
2022
University of Rochester Medical Center
2015-2016
Fondazione Salvatore Maugeri
2014
Drexel University
1988
California University of Pennsylvania
1988
Aging is usually accompanied by a significant reduction in muscle mass and force. To determine the relative contribution of inactivity aging per se to this decay, we compared function structure (a) male participants belonging group well-trained seniors (average 70 years) who exercised regularly their previous 30 years (b) age-matched healthy sedentary with (c) active young men 27 years). The results collected show that cohorts, from senior sportsmen have: greater maximal isometric force...
The loss in muscle mass coupled with a decrease specific force and shift fiber composition are all marks of aging. Training regular exercise attenuate the signs sarcopenia. However, pathologic conditions limit ability to perform physical exercise. We addressed whether electrical stimulation (ES) is an alternative intervention improve recovery defined molecular mechanism associated improvement structure function. analyzed, at functional, structural, level, effects ES training on healthy...
Background— Catecholaminergic polymorphic ventricular tachycardia is an inherited arrhythmogenic disorder characterized by sudden cardiac death in children. Drug therapy still insufficient to provide full protection against arrest, and the use of implantable defibrillators pediatric population limited side effects. There therefore a need explore curative potential gene for this disease. We investigated efficacy durability viral transfer calsequestrin 2 ( CASQ2 ) wild-type catecholaminergic...
// Laura Pietrangelo 1,* , Alessandra D'Incecco Alina Ainbinder 2 Antonio Michelucci 1 Helmut Kern 3 Robert T. Dirksen Simona Boncompagni and Feliciano Protasi CeSI - Center for Research on Aging & DNICS, Department of Neuroscience, Imaging Clinical Sciences, University G. d'Annunzio, Chieti, Italy Pharmacology Physiology, Rochester School Medicine Dentistry, Rochester, NY, USA Ludwig Boltzmann Institute Electrical Stimulation Physical Rehabilitation Rehabilitation, Wilhelminenspital,...
Abstract Age‐related sarcopenia is characterized by a progressive loss of muscle mass with decline in specific force, having dramatic consequences on mobility and quality life seniors. The etiology multifactorial underlying mechanisms are currently not fully elucidated. Physical exercise known to have beneficial effects trophism force production. Alterations mitochondrial Ca 2+ homeostasis regulated calcium uniporter ( MCU ) been recently shown affect vivo mice. To understand the relevance...
Store-operated Ca2+ entry (SOCE), a ubiquitous mechanism that allows recovery of ions from the extracellular space, has been proposed to limit fatigue during repetitive skeletal muscle activity. However, subcellular location for SOCE in fibers not unequivocally identified. Here we show exercise drives significant remodeling sarcotubular system form previously unidentified junctions between sarcoplasmic reticulum (SR) and transverse-tubules (TTs). We also demonstrate these new SR-TT contain...
Neuromuscular junction (NMJ) represents the morphofunctional interface between muscle and nerve. Several chronic pathologies such as aging neurodegenerative diseases, including muscular dystrophy amyotrophic lateral sclerosis, display altered NMJ functional denervation. However, triggers molecular mechanisms underlying dismantlement of remain unclear.Here we provide evidence that perturbation in redox signaling cascades, induced by muscle-specific accumulation mutant SOD1G93A transgenic...
Exercise promotes the formation of intracellular junctions in skeletal muscle between stacks sarcoplasmic reticulum (SR) cisternae and extensions transverse-tubules (TT) that increase co-localization proteins required for store-operated Ca2+ entry (SOCE). Here, we report SOCE, peak transient amplitude force production during repetitive stimulation are increased after exercise parallel with time course TT association SR-stacks. Unexpectedly, also activated constitutive coincident a modest...
Store-operated Ca2+ entry (SOCE) is a ubiquitous influx mechanism triggered by depletion of stores from the endoplasmic/sarcoplasmic reticulum (ER/SR). We recently reported that acute exercise in WT mice drives formation units (CEUs), intracellular junctions contain STIM1 and Orai1, two key proteins mediating SOCE. The presence CEUs correlates with increased constitutive- store-operated entry, as well sustained release force generation during repetitive stimulation. Skeletal muscle lacking...
Tubular aggregates (TAs) in skeletal muscle fibers are unusual accumulation of sarcoplasmic reticulum (SR) tubes that found different disorders including TA myopathy (TAM). TAM is a muscular disease characterized by pain, cramping, and weakness has been recently linked to mutations STIM1 ORAI1. ORAI1 the two main proteins mediating store-operated Ca 2+ entry (SOCE), mechanism activated depletion intracellular stores (e.g., SR) allows recovery from extracellular space during repetitive...
Abstract Background: Mice lacking calsequestrin-1 (CASQ1-null), a Ca2+-binding protein that modulates the activity of Ca2+ release in skeletal muscle, exhibit lethal hypermetabolic episodes resemble malignant hyperthermia humans when exposed to halothane or heat stress. Methods: Because oxidative species may play critical role crises, we treated CASQ1-null mice with two antioxidants, N-acetylcysteine (NAC, Sigma-Aldrich, Italy; provided ad libitum drinking water) and...
Abstract In the last decades term Store-operated Ca 2+ entry (SOCE) has been used in scientific literature to describe an ubiquitous cellular mechanism that allows recovery of calcium (Ca ) from extracellular space. SOCE is triggered by a reduction content (i.e. depletion) intracellular stores, i.e. endoplasmic or sarcoplasmic reticulum (ER and SR). skeletal muscle primarily mediated physical interaction between stromal molecule-1 (STIM1), sensor located SR membrane, ORAI1, -permeable...
Parvalbumin (PV) is a cytosolic Ca2+-binding protein highly expressed in fast skeletal muscle, contributing to an increased relaxation rate. Moreover, PV "atrogene" downregulated most muscle atrophy conditions. Here, we exploit mice lacking explore the link between two functions. Surprisingly, ablation partially counteracts loss after denervation. Furthermore, acute downregulation accompanied by hypertrophy and upregulation atrophy. has minor impact on sarcoplasmic reticulum but associated...
Human umbilical cord endothelial cells (HUVECs) obtained from women affected by gestational diabetes (GD-HUVECs) display durable pro-atherogenic modifications and might be considered a valid in vitro model for studying chronic hyperglycemia effects on early senescence. Here, we demonstrated that GD- compared to C-HUVECs (controls) exhibited oxidative stress, altered both mitochondrial membrane potential antioxidant response, significant increase of senescent characterized reduced...
The skeletal muscle fiber has a specific and precise intracellular organization which is at the basis of an efficient contraction. Microtubules are long known to play major role in function many cells, but muscle, contribution microtubule cytoskeleton efficiency contraction only recently been studied. network dynamic regulated by microtubule-associated proteins (MAPs). In present study, MAP6 protein studied using knockout mouse line. presence transcripts was shown homogenates primary culture...
Recessive ryanodine receptor 1 (RYR1) mutations cause congenital myopathies including multiminicore disease (MmD), fiber-type disproportion and centronuclear myopathy. We created a mouse model knocked-in for the Q1970fsX16+A4329D RYR1 mutations, which are isogenic with those identified in severely affected child MmD. During first 20 weeks after birth body weight spontaneous running distance of mutant mice were 20% 50% lower compared to wild-type littermates. Skeletal muscles from contained...
To date there are no therapies for patients with congenital myopathies, muscle disorders causing poor quality of life affected individuals. In approximately 30% the cases, myopathies carry either dominant or recessive mutations in ryanodine receptor 1 ( RYR1 ) gene; accompanied by reduction RyR1 expression and content skeletal muscles associated fiber hypotrophy weakness. Importantly, exhibit increased class II histone deacetylases DNA genomic methylation. We recently created a mouse model...
RYR1 is the most commonly mutated gene associated with congenital myopathies, a group of early-onset neuromuscular conditions variable severity. The functional effects number dominant mutations have been established; however, for recessive mutations, these may depend on multiple factors, such as formation hypomorphic allele, or whether they are homozygous compound heterozygous. Here, we functionally characterize new transgenic mouse model knocked-in identified in severely affected child born...
Small incision lenticule extraction (SMILE), is a surgical procedure for the myopia correction, during which corneal stromal extracted. Given that we have previously demonstrated how this discarded tissue could be repurposed as bio-scaffold engineering, study aimed to explore its use an ocular drug delivery system of active molecules, using neurotrophic factor Nerve Growth Factor (NGF). We employed human lenticules directly collected from healthy donors undergoing SMILE. Following sodium...
Marinesco–Sjogren syndrome (MSS) is a rare multisystem pediatric disorder, caused by loss-of-function mutations in the gene encoding endoplasmic reticulum cochaperone SIL1. SIL1 acts as nucleotide exchange factor for BiP, which plays central role secretory protein folding. mutant cells have reduced BiP-assisted folding, cannot fulfil their needs, and experience chronic activation of unfolded response (UPR). Maladaptive UPR may explain cerebellar skeletal muscle degeneration responsible...