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Detection of multiple myositis-specific autoantibodies in unique patients with idiopathic inflammatory myopathy: A single centre-experience and literature review: Systematic review.

Semin Arthritis Rheum 2021 Mar 27;51(2):486-494. Epub 2021 Mar 27.

Rheumatology, University Hospitals Leuven, Leuven, Belgium; KU Leuven Department of Development and Regeneration, Skeletal Biology and Engineering Research Centre, Laboratory of Tissue Homeostasis and Disease. Electronic address:

Introduction: Myositis-specific autoantibodies (MSAs) are thought to be mutually exclusive in patients with idiopathic inflammatory myopathies (IIM) based on studies with immunoprecipitation-based (IP) detection methods. Recently, detection of multiple MSAs in unique patients is increasingly reported, but the extent of this phenomenon remains unclear.

Methods: At our centre, we reviewed results from two line immunoassays and one dot immunoassay in 145 IIM patients and 240 controls for the presence of multiple MSAs. Read More

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The pathophysiological effects of exercise in the management of idiopathic inflammatory myopathies: A scoping review.

Int J Rheum Dis 2021 Apr 1. Epub 2021 Apr 1.

Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Idiopathic inflammatory myopathy (IIM) is a term used for a heterogeneous group of diseases characterized by severe muscle weakness. In addition to pharmacological treatment options, non-pharmacological methods such as exercising are essential for proper management of myositis. The present article aimed to provide an insight into the potential pathophysiological mechanisms underlying exercise-related benefits in myositis. Read More

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Clinical characteristics of Vietnamese patients with idiopathic inflammatory myopathies and autoantibodies to aminoacyl-transfer RNA synthetases.

Int J Rheum Dis 2021 Mar 31. Epub 2021 Mar 31.

Division of Rheumatology, Department of Medicine, Karolinska Institutet, Stockholm, Sweden.

Objective: To assess clinical phenotypes of anti-aminoacyl-transfer RNA synthetases (aaRS) autoantibodies in Vietnamese patients of Kinh ethnicity with idiopathic inflammatory myopathies (IIM).

Methods: In a cross-sectional study 23 patients with anti-aaRS autoantibodies were compared to 36 patients with other myositis-specific antibodies and to 69 seronegative patients with IIM. Assessments included muscle performance, extra-muscular involvement, and disease activity according to the International Myositis Assessment and Clinical Studies (IMACS). Read More

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Multiple neurological manifestations in a patient with systemic lupus erythematosus and anti-NXP2-positive myositis: A case report.

Medicine (Baltimore) 2021 Mar;100(10):e25063

Department of Neurology, The General Hospital of Western Theater Command, Jinniu District, Chengdu, Sichuan Province, People's Republic of China.

Rationale: Systemic lupus erythematosus (SLE) is a complex autoimmune inflammatory disease that frequently affects various organs. Neuropsychiatric manifestations in SLE patients, known as neuropsychiatric SLE, are clinically common. However, the principal manifestation of cranial neuropathy in patients with SLE and comorbidities is relatively rare. Read More

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Reliability, Validity and Responsiveness of Physical Activity Monitors in Patients with Inflammatory Myopathy.

Rheumatology (Oxford) 2021 Mar 14. Epub 2021 Mar 14.

Division of Rheumatology and Clinical Immunology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

Objective: Idiopathic inflammatory myopathies (IIM) cause proximal muscle weakness, which affect activities of daily living. Wearable physical activity monitors (PAMs) objectively assess continuous activity with potential clinical usefulness in IIM assessment. We examined the psychometric characteristics for PAM outcomes in IIM. Read More

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Predictors, outcome and characteristics of oropharyngeal dysphagia in idiopathic inflammatory myopathy.

Muscle Nerve 2021 Mar 12. Epub 2021 Mar 12.

Department of Neurology with Institute of Translational Neurology, University of Muenster, Muenster, Germany.

Introduction: Oropharyngeal dysphagia is a clinical hallmark of idiopathic inflammatory myopathy (IIM). This study investigated predictors, outcome, and characteristics of oropharyngeal dysphagia in patients with different types of IIM.

Methods: Flexible endoscopic evaluation of swallowing (FEES) videos of 71 IIM patients were retrospectively analyzed for bolus spillage, penetration, aspiration, and pharyngeal residue. Read More

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FTSC-Labeling Coupled with 2DE-LC-MS/MS Analysis of Complex Protein Mixtures for Identification and Relative Quantification of Tissue Carbonylome.

Methods Mol Biol 2021 ;2259:227-246

Instituto de Investigaciones Marinas-Consejo Superior de Investigaciones Científicas (IIM-CSIC), Vigo, Galicia, Spain.

Carbonylation is a nonenzymatic irreversible posttranslational protein modification and the main hallmark of protein oxidative damage. Elevated levels of protein carbonyl groups have been detected in age-related and metabolic diseases such as obesity, diabetes, Alzheimer, Parkinson, and several other oxidative stress-related maladies. Interestingly, many studies have shown that only a subset of proteins is carbonylated under the conditions of oxidative stress, demonstrating that carbonylation is a highly selective process. Read More

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January 2021

Begelomab for severe refractory dermatomyositis: A case report.

Medicine (Baltimore) 2021 Mar;100(9):e24372

Division of Immunology, Transplantation and Infectious diseases, IRCCS Ospedale San Raffaele.

Rationale: Severe refractory idiopathic inflammatory myopathy (IIM) represents a challenge for the clinician. The lack of efficacy of available tools reflects our incomplete insight into the molecular events sustaining the inflammatory tissue damage in these patients. We present the first case of refractory IIM treated with anti-dipeptidyl peptidase-4 (DPP-4)/cluster of differentiation 26 (CD26) monoclonal antibody. Read More

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Cholecalciferol (vitamin D) has a direct protective activity against interleukin 6-induced atrophy in C2C12 myotubes.

Aging (Albany NY) 2021 02 22;13(4):4895-4910. Epub 2021 Feb 22.

Department of Translational Medicine, University of Piemonte Orientale, Novara, Italy.

We previously determined that different vitamin D metabolites can have opposite effects on C2C12 myotubes, depending on the sites of hydroxylation or doses. Specifically, 25(OH)D (25VD) has an anti-atrophic activity, 1,25(OH)D induces atrophy, and 24,25(OH)D is anti-atrophic at low concentrations and atrophic at high concentrations. This study aimed to clarify whether cholecalciferol (VD3) too, the non-hydroxylated upstream metabolite, has a direct effect on muscle cells. Read More

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February 2021

A Systematic Review and Meta-Analysis to Inform Cancer Screening Guidelines in Idiopathic Inflammatory Myopathies.

Rheumatology (Oxford) 2021 Feb 18. Epub 2021 Feb 18.

Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.

Objectives: To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to cancer screening.

Methods: A systematic literature search was carried out on Medline, Embase and Scopus. Cancer risk within the IIM population (i. Read More

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February 2021

The Role of Immune Cells in the Pathogenesis of Idiopathic Inflammatory Myopathies.

Aging Dis 2021 Feb 1;12(1):247-260. Epub 2021 Feb 1.

1Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, Hunan, China.

Idiopathic inflammatory myopathies (IIMs) are chronic autoimmune disorders involving multiple organs, such as the muscle, skin, lungs and joints. Although the detailed pathogenesis of IIMs remains unclear, immune mechanisms have long been recognised as of key importance. Immune cells contribute to many inflammatory processes via intercellular interactions and secretion of inflammatory factors, and many studies have demonstrated the participation of a variety of immune cells, such as T cells and B cells, in the development of IIMs. Read More

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February 2021

Report and Abstracts of the 17th Meeting of IIM, the Interuniversity Institute of Myology:Virtual meeting, October 16-18, 2020.

Eur J Transl Myol 2020 Dec 14;30(4):9485. Epub 2021 Jan 14.

Gene Expression and Muscular Dystrophy Group, Division of Genetics and Cell Biology, IRCCS San Raffaele Scientific Institute, Milano, Italy.

In 2020, due to the COVID-19 pandemic, the annual meeting of the Interuniversity Institute of Myology (IIM), took place on a virtual platform. Attendees were scientists and clinicians, as well as pharmaceutical companies and patient organization representatives from Italy, several European countries, Canada and USA. Four internationally renowned Keynote speakers presented recent advances on muscle stem cells regulation, skeletal muscle regeneration, quantitative biology approaches, and metabolic regulation of muscle homeostasis. Read More

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December 2020

Systematic review of physical exercise for patients with idiopathic inflammatory myopathies.

Nurs Health Sci 2021 Jan 28. Epub 2021 Jan 28.

School of Nursing, Peking Union Medical College, Beijing, China.

Idiopathic inflammatory myopathies (IIM) are systemic autoimmune connective tissue diseases. The safety and effectiveness of exercise for patients with idiopathic inflammatory myopathies remains unclear. This study aimed to systematically review the evidence for physical exercise among patients with idiopathic inflammatory myopathies. Read More

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January 2021

Mycophenolate in idiopathic inflammatory myositis: outcome data of a large South Asian cohort.

Clin Rheumatol 2021 Jan 27. Epub 2021 Jan 27.

Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital (CMCH), Vellore, Tamil Nadu, 632002, India.

Background: Consensus on treatment of idiopathic inflammatory myositis (IIM), particularly with regard to flares and interstitial lung disease (ILD), does not exist. We studied the long-term outcome and treatment response in our large, retrospective cohort of adult South-Asian patients exclusively with IIM.

Methodology: Electronic records of IIM patients satisfying inclusion and exclusion criteria were studied longitudinally at presentation, at 3, 6, 12, 18 and 24 months and thereafter yearly till their last follow up (F/u) visit. Read More

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January 2021

Real life picture of the use of intravenous immunoglobulins in idiopathic inflammatory myopathies: Results of a multicentric study.

Autoimmun Rev 2021 Mar 18;20(3):102757. Epub 2021 Jan 18.

Rheumatology Unit, Pisa University Hospital, Italy.

Background: despite the absence of specific guidelines, the treatment with intravenous immunoglobulins (IvIg) is considered effective in patients with refractory idiopathic inflammatory myopathies (IIM). The aim of our study is to evaluate the effectiveness and the safety of IvIg and define the possible profile of IIM patients candidate to IvIg treatment.

Methods: we performed a retrospective study of IIM pts. Read More

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The correlation between cardiac and skeletal muscle pathology in animal models of idiopathic inflammatory myopathies.

Acta Myol 2020 Dec 1;39(4):313-319. Epub 2020 Dec 1.

Department of Veterinary Medicine and animal production, Unit of Pathology. University of Naples "Federico II", Naples, Italy.

Idiopathic inflammatory myopathies (IIMs) represent a heterogeneous group of disorders in which skeletal muscle is inappropriately targeted by the immune system. IIMs are characterized by inflammation of muscle and varying degrees of muscle dysfunction. Extra-muscular manifestations may involve heart, skin, joints, lungs, and gastrointestinal tract. Read More

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December 2020

Identification of Formulation as a Nutritional Supplement to Contrast Muscle Atrophy and Sarcopenia.

Nutrients 2020 Dec 26;13(1). Epub 2020 Dec 26.

Interuniversity Institute of Myology (IIM), 06132 Perugia, Italy.

Muscle atrophy, i.e., the loss of skeletal muscle mass and function, is an unresolved problem associated with aging (sarcopenia) and several pathological conditions. Read More

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December 2020

Polymyositis: is there anything left? A retrospective diagnostic review from a tertiary myositis centre.

Rheumatology (Oxford) 2020 Dec 24. Epub 2020 Dec 24.

National Institute for Health Research Manchester Musculoskeletal Biomedical Research Centre, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, University of Manchester, Manchester, UK.

Objective: The current classification criteria for idiopathic inflammatory myopathy (IIM) retain PM as a major disease subgroup. However, evolution in the understanding of IIM has suggested that many of these patients could be better described as having an alternative diagnosis. In the present study, we apply the latest understanding of IIM subtyping to retrospectively review PM diagnoses in a large cohort of IIM patients. Read More

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December 2020

Functional Electrical Stimulation (FES) and the Effect on Equine Multifidi Asymmetry.

J Equine Vet Sci 2020 12 28;95:103255. Epub 2020 Sep 28.

Department of Mathematics, Utah Valley University, Orem, UT.

Asymmetry of the multifidi has been correlated with scoliosis and back pain in humans and has been investigated as a factor in equine back pain as well. The purpose of this study was to determine if FES would affect the symmetry of equine thoracolumbar multifidi when compared to controls. Twelve horses received 24 FES treatments bilaterally over the thoracolumbar region for 8 weeks. Read More

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December 2020

[Myositis: From classification to diagnosis].

Rev Med Interne 2020 Nov 25. Epub 2020 Nov 25.

Département de Médecine interne et immunologie clinique, Centre national de référence des maladies neuromusculaires, hôpital Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.

Idiopathic inflammatory myopathies, or IIM, are a group of acquired diseases that affect the muscle to a certain extent, and may also affect other organs. They include dermatomyositis, which can affect the muscle eventualy, with a typical skin rash; inclusion body myositis, with a purely muscular expression resulting in a slow progressive deficit; and the former group of "polymyositis", a misnomer that actually includes other categories of IIM, such as immune-mediated necrotizing myopathies, with a severe muscle involvement often presents from the onset of the disease; antisynthetase syndrome, which combines muscle damage, joint involvement and a potentially life-threatening lung disease; and overlapping myositis, which combines muscle damage with other organs involvement connected to another autoimmune disease. The diagnosis of IIM is based on rigorous clinical examination and interrogation, electromyographic data and immunological testing for myositis specific antibodies. Read More

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November 2020

Utilization of myositis antibody-specific panel for diagnosis, treatment, and evaluation of disease progression.

J Community Hosp Intern Med Perspect 2020 Sep 3;10(5):462-465. Epub 2020 Sep 3.

Greater Baltimore Medical Center, Internal Medicine Residency, Towson, MD, USA.

The idiopathic inflammatory myopathies (IIM) are rare sporadic disorders with an overall annual incidence of approximately 1 in 100,000 and with a higher incidence in women. IIM is an autoimmune process leading to muscle inflammation due to a 'dysfunctional adaptive immune response evidenced by cell-mediated myocytoxicity, a high prevalence of autoantibodies and overexpression of Major Histocompatibility (MHC) I and II molecules on the muscle sarcolemma'. These autoimmune processes can be appreciated as inflammatory infiltrates in muscle biopsies. Read More

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September 2020

Label-free based proteomics analysis of protein changes in frozen whiteleg shrimp (Litopenaeus vannamei) pre-soaked with sodium trimetaphosphate.

Food Res Int 2020 11 17;137:109455. Epub 2020 Jun 17.

Consejo Superior de Investigaciones Cientificas (CSIC), Inst Invest Marinas, Spain. Electronic address:

Muscle proteins in peeled shrimp (Litopenaeus vannamei) are known to be unstable and prone to denaturation affected by freezing and frozen storage. In this study, label-free proteomics were performed to explore the stabilization of frozen (30 days at -18 °C) shrimp muscle proteins when a pre-soaking treatment with distilled water (DW)- or sodium trimetaphosphate (ST) was applied; comparison to fresh samples (FS) was carried out. In total, 163 differentially abundant proteins (DAPs) were down-regulated in DW batch when compared to FS, these including ribosomal proteins, actins, myosin, paramyosin, myosin heavy chains, and tropomyosin; interestingly, most of these DAPs (181 proteins) were up-regulated in ST batch when compared to DW shrimp, mainly due to the incorporation of ST into muscle tissues. Read More

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November 2020

What is New in Idiopathic Inflammatory Myopathies: Mechanisms and Therapies.

Ann Indian Acad Neurol 2020 Jul-Aug;23(4):458-467. Epub 2020 Apr 13.

Department of Neurology, Dr. L H Hiranandani Hospital, Mumbai, Maharashtra, India.

Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of disorders that cause muscle weakness and also have extramuscular manifestations involving various organ systems; namely the lung, skin, heart, and joints. Previously classified broadly as dermatomyositis (DM) and polymyositis now the spectrum of the disease has evolved into more clinical subtypes. There are now five clinicoserological subtypes recognized worldwide DM, antisynthetase syndrome (AS), overlap myositis (OM), immune mediated necrotizing myopathy (IMNM), and inclusion body myositis. Read More

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Idiopathic inflammatory myopathy human derived cells retain their ability to increase mitochondrial function.

PLoS One 2020 20;15(11):e0242443. Epub 2020 Nov 20.

Geroscience Center for Brain Health and Metabolism, Santiago, Chile.

Idiopathic Inflammatory Myopathies (IIMs) have been studied within the framework of autoimmune diseases where skeletal muscle appears to have a passive role in the illness. However, persiting weakness even after resolving inflammation raises questions about the role that skeletal muscle plays by itself in these diseases. "Non-immune mediated" hypotheses have arisen to consider inner skeletal muscle cell processes as trigger factors in the clinical manifestations of IIMs. Read More

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January 2021

Eccentric Resistance Training Ameliorates Muscle Weakness in a Mouse Model of Idiopathic Inflammatory Myopathies.

Arthritis Rheumatol 2020 Nov 15. Epub 2020 Nov 15.

Sapporo Medical University, Sapporo, Japan.

Objective: High-force eccentric contractions (ECCs) have traditionally been excluded from rehabilitation programs that include patients with idiopathic inflammatory myopathies (IIMs) due to unverified fear of causing muscle damage and inflammation. In an IIM animal model that used mice with experimental autoimmune myositis (EAM), we undertook this study to investigate whether ECC training can safely and effectively be used to counteract muscle weakness in IIM.

Methods: EAM was induced in BALB/c mice by immunization with 3 injections of myosin emulsified in Freund's complete adjuvant. Read More

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November 2020

Cricopharyngeal bar and dermatomyositis: A cause of rapidly progressive dysphagia.

Int J Rheum Dis 2021 Jan 1;24(1):125-131. Epub 2020 Nov 1.

Private Rheumatology Practice, Campbelltown, NSW, Australia.

Background: Idiopathic inflammatory myopathies (IIM) are immune-mediated conditions that affect striated muscle, and are frequently associated with dysphagia. Dysphagia in these cases can be due to weakness of the muscles involved in swallowing or the presence of restrictive pharyngeal defects, such as cricopharyngeal bars. Treatment of dysphagia in IIM revolves around immunosuppressive therapies, and procedures to disrupt cricopharyngeus muscle when immunosuppressive therapies are unsuccessful. Read More

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January 2021

Prognostic factors for steroid-free remission in patients with idiopathic inflammatory myopathies: importance of anthropometric measurements.

Ther Adv Musculoskelet Dis 2020 16;12:1759720X20936822. Epub 2020 Oct 16.

Division of Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Centre, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea.

Background: Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIMs), but few studies have assessed prognostic factors for steroid-free remission in IIM. We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes.

Methods: Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Read More

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October 2020

Interleukin-35 in idiopathic inflammatory myopathies.

Cytokine 2021 Jan 28;137:155350. Epub 2020 Oct 28.

Institute of Rheumatology and Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic.

Background: Interleukin-35 (IL-35) is a recently described heterodimeric cytokine that belongs to the IL-12 family and consists of p35 (IL-12a) and EBI3 (IL-27b) subunits. The expression of IL-35 in humans is inducible in response to inflammatory stimuli. Increased IL-35 levels were documented in several autoimmune inflammatory diseases, suggesting a possible immunomodulatory role in their pathogenesis. Read More

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January 2021

Clusterin is upregulated in serum and muscle tissue in idiopathic inflammatory myopathies and associates with clinical disease activity and cytokine profile.

Clin Exp Rheumatol 2020 Oct 29. Epub 2020 Oct 29.

Institute of Rheumatology, Prague, and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic.

Objectives: The aim of this cross-sectional study was to explore the circulating and skeletal muscle expression of clusterin (CLU) in inflammatory myopathies (IIM) and its potential implication in pathogenetic mechanisms of the disease.

Methods: A total of 85 IIM patients and 86 healthy controls (HC) were recruited. In addition, 20 IIM patients and 21 HC underwent a muscle biopsy. Read More

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October 2020

16th Meeting of the Interuniversity Institute of Myology (IIM) - Assisi (Italy), October 17-20, 2019: Foreword, Program and Abstracts.

Eur J Transl Myol 2020 Sep 15;30(3):9345. Epub 2020 Sep 15.

DAHFMO-Unit of Histology and Medical Embryology, Laboratory Affiliated to "Istituto Pasteur Italia - Fondazione Cenci Bolognetti", Sapienza University of Rome, Rome, Italy.

The 16th Meeting of the Interuniversity Institute of Myology (IIM), October 17-20, 2019, Assisi, Italy brought together scientists, pharma and patient organization representatives discussing new results on muscle research. Internationally renowned Keynote speakers presented advances on muscle development, homeostasis, metabolism, and disease. Speakers selected among submitted abstracts presented their new, unpublished data in seven scientific sessions. Read More

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September 2020