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Article ; Online: Preclinical Ultrasonography in Rodent Models of Neuromuscular Disorders: The State of the Art for Diagnostic and Therapeutic Applications.

Mele, Antonietta / Mantuano, Paola / Boccanegra, Brigida / Conte, Elena / Liantonio, Antonella / De Luca, Annamaria

International journal of molecular sciences

2023 Volume 24, Issue 5

Abstract: Ultrasonography is a safe, non-invasive imaging technique used in several fields of medicine, offering the possibility to longitudinally monitor disease progression and treatment efficacy over time. This is particularly useful when a close follow-up is ... ...

Abstract	Ultrasonography is a safe, non-invasive imaging technique used in several fields of medicine, offering the possibility to longitudinally monitor disease progression and treatment efficacy over time. This is particularly useful when a close follow-up is required, or in patients with pacemakers (not suitable for magnetic resonance imaging). By virtue of these advantages, ultrasonography is commonly used to detect multiple skeletal muscle structural and functional parameters in sports medicine, as well as in neuromuscular disorders, e.g., myotonic dystrophy and Duchenne muscular dystrophy (DMD). The recent development of high-resolution ultrasound devices allowed the use of this technique in preclinical settings, particularly for echocardiographic assessments that make use of specific guidelines, currently lacking for skeletal muscle measurements. In this review, we describe the state of the art for ultrasound skeletal muscle applications in preclinical studies conducted in small rodents, aiming to provide the scientific community with necessary information to support an independent validation of these procedures for the achievement of standard protocols and reference values useful in translational research on neuromuscular disorders.
MeSH term(s)	Humans ; Muscle, Skeletal ; Neuromuscular Diseases ; Muscular Dystrophy, Duchenne ; Ultrasonography ; Echocardiography
Language	English
Publishing date	2023-03-04
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2019364-6
ISSN	1422-0067 ; 1422-0067 ; 1661-6596
ISSN (online)	1422-0067
ISSN	1422-0067 ; 1661-6596
DOI	10.3390/ijms24054976
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Blockers of Skeletal Muscle Na

De Bellis, Michela / Boccanegra, Brigida / Cerchiara, Alessandro Giovanni / Imbrici, Paola / De Luca, Annamaria

International journal of molecular sciences

2023 Volume 24, Issue 1

Abstract: The voltage-gated sodium channels represent an important target for drug discovery since a large number of physiological processes are regulated by these channels. In several excitability disorders, including epilepsy, cardiac arrhythmias, chronic pain, ... ...

Abstract	The voltage-gated sodium channels represent an important target for drug discovery since a large number of physiological processes are regulated by these channels. In several excitability disorders, including epilepsy, cardiac arrhythmias, chronic pain, and non-dystrophic myotonia, blockers of voltage-gated sodium channels are clinically used. Myotonia is a skeletal muscle condition characterized by the over-excitability of the sarcolemma, resulting in delayed relaxation after contraction and muscle stiffness. The therapeutic management of this disorder relies on mexiletine and other sodium channel blockers, which are not selective for the Na
MeSH term(s)	Humans ; Mexiletine/pharmacology ; Mexiletine/therapeutic use ; Muscle, Skeletal/drug effects ; Myotonia/drug therapy ; NAV1.4 Voltage-Gated Sodium Channel/metabolism ; Syndrome ; Voltage-Gated Sodium Channel Blockers/pharmacology ; Voltage-Gated Sodium Channel Blockers/therapeutic use
Chemical Substances	Mexiletine (1U511HHV4Z) ; NAV1.4 Voltage-Gated Sodium Channel ; Voltage-Gated Sodium Channel Blockers
Language	English
Publishing date	2023-01-03
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2019364-6
ISSN	1422-0067 ; 1422-0067 ; 1661-6596
ISSN (online)	1422-0067
ISSN	1422-0067 ; 1661-6596
DOI	10.3390/ijms24010857
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Article ; Online: Myasthenia Gravis Treatment: From Old Drugs to Innovative Therapies with a Glimpse into the Future.

Crisafulli, Salvatore / Boccanegra, Brigida / Carollo, Massimo / Bottani, Emanuela / Mantuano, Paola / Trifirò, Gianluca / De Luca, Annamaria

CNS drugs

2024 Volume 38, Issue 1, Page(s) 15–32

Abstract: Myasthenia gravis (MG) is a rare autoimmune disease that causes debilitating muscle weakness due to impaired neuromuscular transmission. Since most (about 80-90%) MG patients present autoantibodies against the acetylcholine receptor, standard medical ... ...

Abstract	Myasthenia gravis (MG) is a rare autoimmune disease that causes debilitating muscle weakness due to impaired neuromuscular transmission. Since most (about 80-90%) MG patients present autoantibodies against the acetylcholine receptor, standard medical therapy consists of symptomatic treatment with acetylcholinesterase inhibitors (e.g., pyridostigmine). In addition, considering the autoimmune basis of MG, standard therapy includes immunomodulating agents, such as corticosteroids, azathioprine, cyclosporine A, and cyclophosphamide. New strategies have been proposed for the treatment of MG and include complement blockade (i.e., eculizumab, ravulizumab, and zilucoplan) and neonatal Fc receptor antagonism (i.e., efgartigimod and rozanolixizumab). The aim of this review is to provide a detailed overview of the pre- and post-marketing evidence on the five pharmacological treatments most recently approved for the treatment of MG, by identifying both preclinical and clinical studies registered in clinicaltrials.gov. A description of the molecules currently under evaluation for the treatment of MG is also provided.
MeSH term(s)	Humans ; Infant, Newborn ; Acetylcholinesterase/therapeutic use ; Adrenal Cortex Hormones/therapeutic use ; Autoantibodies ; Myasthenia Gravis/drug therapy ; Receptors, Cholinergic/therapeutic use ; Therapies, Investigational
Chemical Substances	Acetylcholinesterase (EC 3.1.1.7) ; Adrenal Cortex Hormones ; Autoantibodies ; Receptors, Cholinergic
Language	English
Publishing date	2024-01-11
Publishing country	New Zealand
Document type	Journal Article ; Review
ZDB-ID	1203800-3
ISSN	1179-1934 ; 1172-7047
ISSN (online)	1179-1934
ISSN	1172-7047
DOI	10.1007/s40263-023-01059-8
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Article ; Online: Ion channels as biomarkers of altered myogenesis in myofiber precursors of Duchenne muscular dystrophy.

Cerchiara, Alessandro Giovanni / Imbrici, Paola / Quarta, Raffaella / Cristiano, Enrica / Boccanegra, Brigida / Caputo, Erika / Wells, Dominic J / Cappellari, Ornella / De Luca, Annamaria

Annals of the New York Academy of Sciences

2024 Volume 1534, Issue 1, Page(s) 130–144

Abstract: Myogenesis is essential for skeletal muscle formation, growth, and regeneration and can be altered in Duchenne muscular dystrophy (DMD), an X-linked disorder due to the absence of the cytoskeletal protein dystrophin. Ion channels play a pivotal role in ... ...

Abstract	Myogenesis is essential for skeletal muscle formation, growth, and regeneration and can be altered in Duchenne muscular dystrophy (DMD), an X-linked disorder due to the absence of the cytoskeletal protein dystrophin. Ion channels play a pivotal role in muscle differentiation and interact with the dystrophin complex. To investigate ion channel involvement in myogenesis in dystrophic settings, we performed electrophysiological characterization of two immortalized mouse cell lines, wild-type (WT) H2K-2B4 and the dystrophic (DYS) H2K-SF1, and measured gene expression of differentiation markers and ion channels. Inward and outward currents/density increased as differentiation progressed in both WT and DYS cells. However, day-11 DYS cells showed higher (27%) inward current density with an increased expression ratio of Scn5a/Scn4a and decreased (48%) barium-sensitive outward current compared to WT. Furthermore, day-11 DYS cells showed more positive resting membrane potential (+10 mV) and lower membrane capacitance (50%) compared to WT. DYS cells also had reduced Myog and Myf5 expression at days 6 and 11. Overall, ion channel profile and myogenesis appeared altered in DYS cells. These results are a first step in validating ion channels as potential drug targets to ameliorate muscle degeneration in DMD settings and as differentiation biomarkers in innovative platforms.
MeSH term(s)	Animals ; Mice ; Muscular Dystrophy, Duchenne/metabolism ; Dystrophin/metabolism ; Muscle, Skeletal/metabolism ; Biomarkers/metabolism ; Ion Channels/metabolism ; Muscle Development
Chemical Substances	Dystrophin ; Biomarkers ; Ion Channels
Language	English
Publishing date	2024-03-22
Publishing country	United States
Document type	Journal Article
ZDB-ID	211003-9
ISSN	1749-6632 ; 0077-8923
ISSN (online)	1749-6632
ISSN	0077-8923
DOI	10.1111/nyas.15124
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Article: Pharmacological Therapies of Spinal Muscular Atrophy: A Narrative Review of Preclinical, Clinical-Experimental, and Real-World Evidence.

Crisafulli, Salvatore / Boccanegra, Brigida / Vitturi, Giacomo / Trifirò, Gianluca / De Luca, Annamaria

Brain sciences

2023 Volume 13, Issue 10

Abstract: Spinal muscular atrophy (SMA) is a rare neuromuscular disease, with an estimated incidence of about 1 in 10,000 live births. To date, three orphan drugs have been approved for the treatment of SMA: nusinersen, onasemnogene abeparvovec, and risdiplam. The ...

Abstract	Spinal muscular atrophy (SMA) is a rare neuromuscular disease, with an estimated incidence of about 1 in 10,000 live births. To date, three orphan drugs have been approved for the treatment of SMA: nusinersen, onasemnogene abeparvovec, and risdiplam. The aim of this narrative review was to provide an overview of the pre- and post-marketing evidence on the pharmacological treatments approved for the treatment of SMA by identifying preclinical and clinical studies registered in clinicaltrials.gov and in the EU PAS register from their inception until the 4 January 2023. The preclinical evidence on the drugs approved for SMA allowed a significant acceleration in the experimental phase of these drugs. However, since these drugs had been authorized through accelerated programs, the conduction of post-marketing studies was requested as a condition of their marketing approval to better understand their risk-benefit profiles in real-world settings. As of the 4 January 2023, a total of 69 post-marketing studies concerning the three orphan drugs approved for SMA were identified in clinicaltrials.gov (N = 65; 94.2%) and in the EU PAS register (N = 4; 5.8%). Currently, ongoing studies are primarily aimed at providing evidence concerning the risk-benefit profile of the three drugs in specific populations that were not included in the pivotal trials and to investigate the long-term safety and clinical benefits of these drugs. Real-world data sources collecting information regarding the natural history of the disease and post-marketing surveillance of the available therapies are increasingly becoming essential for generating real-world evidence on this rare disease and its orphan drugs.
Language	English
Publishing date	2023-10-10
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2651993-8
ISSN	2076-3425
ISSN	2076-3425
DOI	10.3390/brainsci13101446
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Article ; Online: Preclinical Ultrasonography in Rodent Models of Neuromuscular Disorders

Antonietta Mele / Paola Mantuano / Brigida Boccanegra / Elena Conte / Antonella Liantonio / Annamaria De Luca

International Journal of Molecular Sciences, Vol 24, Iss 4976, p

The State of the Art for Diagnostic and Therapeutic Applications

2023 Volume 4976

Abstract	Ultrasonography is a safe, non-invasive imaging technique used in several fields of medicine, offering the possibility to longitudinally monitor disease progression and treatment efficacy over time. This is particularly useful when a close follow-up is required, or in patients with pacemakers (not suitable for magnetic resonance imaging). By virtue of these advantages, ultrasonography is commonly used to detect multiple skeletal muscle structural and functional parameters in sports medicine, as well as in neuromuscular disorders, e.g., myotonic dystrophy and Duchenne muscular dystrophy (DMD). The recent development of high-resolution ultrasound devices allowed the use of this technique in preclinical settings, particularly for echocardiographic assessments that make use of specific guidelines, currently lacking for skeletal muscle measurements. In this review, we describe the state of the art for ultrasound skeletal muscle applications in preclinical studies conducted in small rodents, aiming to provide the scientific community with necessary information to support an independent validation of these procedures for the achievement of standard protocols and reference values useful in translational research on neuromuscular disorders.
Keywords	ultrasonography ; skeletal muscle ; neuromuscular disorders ; translational research ; Biology (General) ; QH301-705.5 ; Chemistry ; QD1-999
Subject code	796
Language	English
Publishing date	2023-03-01T00:00:00Z
Publisher	MDPI AG
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article ; Online: Blockers of Skeletal Muscle Na v 1.4 Channels

Michela De Bellis / Brigida Boccanegra / Alessandro Giovanni Cerchiara / Paola Imbrici / Annamaria De Luca

International Journal of Molecular Sciences, Vol 24, Iss 1, p

From Therapy of Myotonic Syndrome to Molecular Determinants of Pharmacological Action and Back

2023 Volume 857

Abstract	The voltage-gated sodium channels represent an important target for drug discovery since a large number of physiological processes are regulated by these channels. In several excitability disorders, including epilepsy, cardiac arrhythmias, chronic pain, and non-dystrophic myotonia, blockers of voltage-gated sodium channels are clinically used. Myotonia is a skeletal muscle condition characterized by the over-excitability of the sarcolemma, resulting in delayed relaxation after contraction and muscle stiffness. The therapeutic management of this disorder relies on mexiletine and other sodium channel blockers, which are not selective for the Na v 1.4 skeletal muscle sodium channel isoform. Hence, the importance of deepening the knowledge of molecular requirements for developing more potent and use-dependent drugs acting on Na v 1.4. Here, we review the available treatment options for non-dystrophic myotonia and the structure–activity relationship studies performed in our laboratory with a focus on new compounds with potential antimyotonic activity.
Keywords	sodium channel ; skeletal muscle ; myotonia ; mexiletine ; SAR ; Biology (General) ; QH301-705.5 ; Chemistry ; QD1-999
Language	English
Publishing date	2023-01-01T00:00:00Z
Publisher	MDPI AG
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article ; Online: LKB1 signaling is altered in skeletal muscle of a Duchenne muscular dystrophy mouse model

Brigida Boccanegra / Paola Mantuano / Elena Conte / Alessandro Giovanni Cerchiara / Lisamaura Tulimiero / Raffaella Quarta / Erika Caputo / Francesca Sanarica / Monica Forino / Valeria Spadotto / Ornella Cappellari / Gianluca Fossati / Christian Steinkühler / Annamaria De Luca

Disease Models & Mechanisms, Vol 16, Iss

2023 Volume 7

Keywords	duchenne muscular dystrophy ; lkb1 ; ampk ; epigenetic ; muscle metabolism ; mouse models ; Medicine ; R ; Pathology ; RB1-214
Language	English
Publishing date	2023-07-01T00:00:00Z
Publisher	The Company of Biologists
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article ; Online: LKB1 signaling is altered in skeletal muscle of a Duchenne muscular dystrophy mouse model.

Boccanegra, Brigida / Mantuano, Paola / Conte, Elena / Cerchiara, Alessandro Giovanni / Tulimiero, Lisamaura / Quarta, Raffaella / Caputo, Erika / Sanarica, Francesca / Forino, Monica / Spadotto, Valeria / Cappellari, Ornella / Fossati, Gianluca / Steinkühler, Christian / De Luca, Annamaria

Disease models & mechanisms

2023 Volume 16, Issue 7

Abstract: The potential role of liver kinase B1 (LKB1) in the altered activation of the master metabolic and epigenetic regulator adenosine monophosphate-activated protein kinase (AMPK) in Duchenne muscular dystrophy has not been investigated so far. Hence, we ... ...

Abstract	The potential role of liver kinase B1 (LKB1) in the altered activation of the master metabolic and epigenetic regulator adenosine monophosphate-activated protein kinase (AMPK) in Duchenne muscular dystrophy has not been investigated so far. Hence, we analyzed both gene and protein levels of LKB1 and its related targets in gastrocnemius muscles of adult C57BL/10 mdx mice and D2 mdx mice, a model with a more severe dystrophic phenotype, as well as the sensitivity of the LKB1-AMPK pathway to AMPK activators, such as chronic exercise. Our data show, for the first time, a reduction in the levels of LKB1 and accessory proteins, MO25 and STRADα, in both mdx strains versus the respective wild type, which was further impaired by exercise, in parallel with a lack of further phosphorylation of AMPK. The AMPK-like kinase salt-inducible kinase (SIK) and class II histone deacetylases, along with expression of the HDAC target gene Mef2c, were also altered, supporting an impairment of LKB1-SIK-class II histone deacetylase signaling. Our results demonstrate that LKB1 may be involved in dystrophic progression, paving the way for future preclinical studies.
MeSH term(s)	Animals ; Mice ; AMP-Activated Protein Kinases/metabolism ; Disease Models, Animal ; Mice, Inbred C57BL ; Mice, Inbred mdx ; Muscle, Skeletal/metabolism ; Muscular Dystrophy, Duchenne/genetics ; Muscular Dystrophy, Duchenne/metabolism ; Protein Serine-Threonine Kinases/metabolism
Chemical Substances	AMP-Activated Protein Kinases (EC 2.7.11.31) ; Protein Serine-Threonine Kinases (EC 2.7.11.1) ; Stk11 protein, mouse (EC 2.7.11.1)
Language	English
Publishing date	2023-07-10
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2451104-3
ISSN	1754-8411 ; 1754-8403
ISSN (online)	1754-8411
ISSN	1754-8403
DOI	10.1242/dmm.049930
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Branched-Chain Amino Acids and Di-Alanine Supplementation in Aged Mice: A Translational Study on Sarcopenia.

Mantuano, Paola / Boccanegra, Brigida / Bianchini, Gianluca / Cappellari, Ornella / Tulimiero, Lisamaura / Conte, Elena / Cirmi, Santa / Sanarica, Francesca / De Bellis, Michela / Mele, Antonietta / Liantonio, Antonella / Allegretti, Marcello / Aramini, Andrea / De Luca, Annamaria

Nutrients

2023 Volume 15, Issue 2

Abstract: In age-related sarcopenia, the gradual loss of skeletal muscle mass, function and strength is underpinned by an imbalanced rate of protein synthesis/breakdown. Hence, an adequate protein intake is considered a valuable strategy to mitigate sarcopenia. ... ...

Abstract	In age-related sarcopenia, the gradual loss of skeletal muscle mass, function and strength is underpinned by an imbalanced rate of protein synthesis/breakdown. Hence, an adequate protein intake is considered a valuable strategy to mitigate sarcopenia. Here, we investigated the effects of a 12-week oral supplementation with branched-chain amino acids (BCAAs: leucine, isoleucine, and valine) with recognized anabolic properties, in 17-month-old (AGED) C57BL/6J male mice. BCAAs (2:1:1) were formulated in drinking water, alone or plus two L-Alanine equivalents (2ALA) or dipeptide L-Alanyl-L-Alanine (Di-ALA) to boost BCAAs bioavailability. Outcomes were evaluated on in/ex vivo readouts vs. 6-month-old (ADULT) mice. In vivo hind limb plantar flexor torque was improved in AGED mice treated with BCAAs + Di-ALA or 2ALA (recovery score, R.S., towards ADULT: ≥20%), and all mixtures significantly increased hind limb volume. Ex vivo, myofiber cross-sectional areas were higher in gastrocnemius (GC) and soleus (SOL) muscles from treated mice (R.S. ≥ 69%). Contractile indices of isolated muscles were improved by the mixtures, especially in SOL muscle (R.S. ≥ 20%). The latter displayed higher mTOR protein levels in mice supplemented with 2ALA/Di-ALA-enriched mixtures (R.S. ≥ 65%). Overall, these findings support the usefulness of BCAAs-based supplements in sarcopenia, particularly as innovative formulations potentiating BCAAs bioavailability and effects.
MeSH term(s)	Male ; Mice ; Animals ; Amino Acids, Branched-Chain/metabolism ; Sarcopenia/metabolism ; Mice, Inbred C57BL ; Muscle, Skeletal/metabolism ; Dietary Supplements
Chemical Substances	Amino Acids, Branched-Chain
Language	English
Publishing date	2023-01-09
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2518386-2
ISSN	2072-6643 ; 2072-6643
ISSN (online)	2072-6643
ISSN	2072-6643
DOI	10.3390/nu15020330
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