scholarly journals High degree of conservation of the enzymes synthesizing the laminin-binding glycoepitope of α-dystroglycan

Open Biology ◽  
2021 ◽  
Vol 11 (9) ◽  
Author(s):  
Maria Giulia Bigotti ◽  
Andrea Brancaccio
Keyword(s):  
Sequence Comparison ◽  
Neuromuscular Disorders ◽  
Basement Membranes ◽  
Evolutionary Perspective ◽  
Carbohydrate Structure ◽  
Comparison Approach ◽  
Binding Epitope ◽  
History Of ◽  
High Degree ◽  
Laminin Binding

The dystroglycan (DG) complex plays a pivotal role for the stabilization of muscles in Metazoa. It is formed by two subunits, extracellular α-DG and transmembrane β-DG, originating from a unique precursor via a complex post-translational maturation process. The α-DG subunit is extensively glycosylated in sequential steps by several specific enzymes and employs such glycan scaffold to tightly bind basement membrane molecules. Mutations of several of these enzymes cause an alteration of the carbohydrate structure of α-DG, resulting in severe neuromuscular disorders collectively named dystroglycanopathies. Given the fundamental role played by DG in muscle stability, it is biochemically and clinically relevant to investigate these post-translational modifying enzymes from an evolutionary perspective. A first phylogenetic history of the thirteen enzymes involved in the fabrication of the so-called ‘M3 core’ laminin-binding epitope has been traced by an overall sequence comparison approach, and interesting details on the primordial enzyme set have emerged, as well as substantial conservation in Metazoa. The optimization along with the evolution of a well-conserved enzymatic set responsible for the glycosylation of α-DG indicate the importance of the glycosylation shell in modulating the connection between sarcolemma and surrounding basement membranes to increase skeletal muscle stability, and eventually support movement and locomotion.

THE R.J. CHAMBERS COLLECTION: AN “ARCHIVIST'S” REVELATIONS OF 20th CENTURY ACCOUNTING THOUGHT AND PRACTICE

2006 ◽  
Vol 33 (1) ◽  
pp. 145-166 ◽  
Author(s):  
Graeme W. Dean ◽  
Peter W. Wolnizer ◽  
Frank L. Clarke
Keyword(s):  
Prima Facie ◽  
Accounting Education ◽  
Hard Copy ◽  
Accounting History ◽  
Research Areas ◽  
Archival Source ◽  
Inflation Accounting ◽  
History Of ◽  
High Degree ◽  
Accounting Standard Setting

A major, unique accounting archival source, the R.J. Chambers Collection comprises both hard copy and, utilizing cutting-edge search technology, internet accessible materials. From his academic beginnings, Chambers was an orderly person, an archivist of the extensive and varied evidence that underpinned his proposals for accounting reform. Opening research areas for accounting biography, the development of accounting thought, the history of accounting institutions, prosopography, public sector accounting history, and comparative international accounting history are foremost amongst the myriad justifications for seeking to unravel the accounting history “lodes” in archives such as the Goldberg, Chambers, and Briloff Collections [Potter, 2003]. The archiving of the meticulously kept Chambers papers from 1947–1999 provides an opportunity for unfolding the background to events previously withheld from accounting history scholars. Professional episodes in relation to inflation accounting, standard setting, proposals to reform accounting education, and the like that appeared prima facie to be worth investigating are now open to scrutiny from a different angle, with a different type of evidence available in this Collection. This Collection provides a high degree of archival provenance. In particular, it represents an orderly retention of past documentation of what Chambers wrote, and perhaps uniquely for accounting historians, received; thus, providing an extensive window from which to examine the disorderly present environment of acounting.

Handbook of Electrogastrography

2003 ◽  
Keyword(s):  
Electrical Activity ◽  
Neuromuscular Disorders ◽  
Relevant Information ◽  
Cellular Level ◽  
Clinical Practices ◽  
Physiological Basis ◽  
Gastric Dysrhythmias ◽  
History Of ◽  
Major Section ◽  
Cells Of Cajal

The Handbook of Electrogastrography is the first textbook dedicated to reviewing the physiology of gastric myelectrical activity and the measurement of this electrical activity with electrodes placed on the abdominal surface - the electrogastrogram. The Handbook is divided into three major sections. The first section (Chapters 1-3) focuses on the history of electrogastrography, electrical activity of the interstitial cells of Cajal, the cells from which gastric electrical rhythmicity emanates. The cellular level of gastric electrical rhythmicity provides an understanding of the physiological basis of the electrogastrogram signal. The second major section of the book (Chapters 4-6) incorporates the practical aspects of recording a high quality electrogastrogram and approaches to the analysis of the electrogastrogram using visual inspection and computer techniques. This section focuses on the authors' combined experience of examining EGG recordings for more than sixty years. From this rich research and clinical experience, the clinical application of EGG recordings in an approach to patients with unexplained nausea and vomiting is described. Neuromuscular disorders of the stomach involving gastric dysrhythmias are reviewed. The third major section of the book (Chapters 7-9) comprises many examples of gastric dysrythmias ranging from bradygastrias to tachygastrias and mixed dysrythmias. Current understanding of the mechanisms of gastric dysrhythmias is reviewed. Artifacts in the EGG signal, which may be confused with gastric dysrhythmias, are also presented. The Handbook of Electrogastrography will be a valuable reference for physicians interested in recording gastric electrical activity in clinical practices or in clinical research. Gastroenterologists, internists, psychologists and others with an interest in gastric myoelectrical events will also find extensive and relevant information for recording and interpreting EGGs in the Handbook.

scholarly journals Euglycemic Diabetic Ketoacidosis after a Single Dose of Empagliflozin in a Patient with Pancreatitis

2021 ◽  
Vol 11 (2) ◽  
pp. 216-218
Author(s):  
Marta Brandão Calçada ◽  
Luís Fernandes ◽  
Rita Soares Costa ◽  
Sara Montezinho ◽  
Filipa Martins Duarte ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Diabetic Ketoacidosis ◽  
Drug Class ◽  
Clinical Suspicion ◽  
Laboratory Findings ◽  
Sodium Glucose Cotransporter ◽  
History Of ◽  
High Degree ◽  
Euglycemic Diabetic Ketoacidosis

Sodium-glucose cotransporter 2 inhibitors (SGLT2i) are the most recently approved drug class for the treatment of type 2 diabetes mellitus (T2D). Although they are largely well-tolerated, their intake has been associated with euglycemic diabetic ketoacidosis (DKA) in some rare cases. We report the case of a 70-year-old male with type 2 diabetes and no history of DKA, who started therapy with empagliflozin one day before presenting with acute pancreatitis and laboratory findings consistent with euglycemic DKA. SGLT2i can induce euglycemic DKA from the first dose. Given the atypical presentation, a high degree of clinical suspicion is required to recognize this complication.

929 Not Another Abscess - A Case of Streptococcal Myositis Misdiagnosed as a Right Axillary Abscess

2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
O Oyende ◽  
J Jackman
Keyword(s):  
White Cell Count ◽  
General Surgeon ◽  
High Dose ◽  
General Anaesthetic ◽  
Microbiological Analysis ◽  
Reactive Protein ◽  
Affected Tissue ◽  
Life Threatening ◽  
History Of ◽  
High Degree

Abstract Introduction Streptococcal myositis is a rare form of infectious myositis caused by Lansfield A beta-haemolytic streptococci. It is characterised by rapidly spreading inflammation that can result in severe systemic toxicity and necrosis of the affected tissue if not diagnosed and aggressively treated. Presentation We report a case of a 42-year-old male who presented with a one-week history of worsening right axillary swelling that progressed to painful swelling of his arm. Inflammatory markers were significantly elevated with a white cell count of 17 ×109/L and C-reactive protein of 212 mg/L. On examination, a fluctuant axillary swelling was appreciated, and a decision was made for incision and drainage under general anaesthetic. Intraoperative aspiration of his arm revealed copious purulent fluid prompting intraoperative orthopaedic consult and exploration of the anterior compartment in which there was extensive involvement of the biceps muscle. The microbiological analysis revealed gram-positive cocci in chains, and microbiology advice sought for tailoring of antibiotic regimen. He has recovered well. Discussion Though uncommon, the emergency general surgeon should have a high degree of suspicion when evaluating soft tissue infections to avert potentially disastrous outcomes. Conclusion Early diagnosis, aggressive management with high-dose intravenous antibiotics, and surgical debridement are principles to treat this rare, life-threatening infection.

scholarly journals The Return of National Self-Sufficiency? Excavating Autarkic Thought in a De-Globalizing Era

2021 ◽  
Author(s):  
Eric Helleiner
Keyword(s):  
Intellectual History ◽  
International Political Economy ◽  
Economic Thought ◽  
John Maynard Keynes ◽  
New Politics ◽  
Self Sufficiency ◽  
National Economic ◽  
History Of ◽  
High Degree ◽  
Rich History

Abstract As the global crisis triggered by the COVID-19 virus unfolded, The Economist magazine published a cover in May 2020 titled “Goodbye globalization: the dangerous lure of self-sufficiency.” The title summed up well the new political interest in the ideology of national economic self-sufficiency in the pandemic context. Unfortunately, contemporary textbooks in the field of international political economy (IPE) say little about this kind of “autarkic” thought. No survey of the history of autarkic thought exists even within specialist IPE literature or in the fields of intellectual history and the history of economic thought. Filling this gap in existing scholarship, this article highlights a rich history of autarkic thought that includes the ideas of famous thinkers such as Jean-Jacques Rousseau, Johann Fichte, Mohandas Gandhi, and John Maynard Keynes. Three core rationales for a high degree of national self-sufficiency have been advanced in the past: (1) insulation from foreign economic influence, (2) insulation from foreign political and/or cultural influence, and (3) the promotion of international peace. At the same time, considerable disagreements have existed among autarkists about some of these rationales and their relative importance, as well as about the precise meaning of national self-sufficiency. These disagreements stemmed not just from differences in their specific goals but also from the different conditions across time and space in which autarkic thought was developed. In addition to improving understanding of the autarkic ideological tradition, this article contributes to emerging scholarship attempting to overcome Western-centrism in IPE scholarship as well as literature exploring the new politics of de-globalization in the current era.

scholarly journals Multi-Locus Selection and the Structure of Variation at the white Gene of Drosophila melanogaster

Genetics ◽  
1996 ◽  
Vol 144 (2) ◽  
pp. 635-645 ◽  
Author(s):  
David A Kirby ◽  
Wolfgang Stephan
Keyword(s):  
Drosophila Melanogaster ◽  
White Gene ◽  
Transcriptional Unit ◽  
North American Population ◽  
Evolutionary Mechanisms ◽  
Locus Selection ◽  
Neutral Equilibrium ◽  
History Of ◽  
High Degree ◽  
Very High

Abstract We surveyed sequence variation and divergence for the entire 5972-bp transcriptional unit of the white gene in 15 lines of Drosophila melanogaster and one line of D. simulans. We found a very high degree of haplotypic structuring for the polymorphisms in the 3′ half of the gene, as opposed to the polymorphisms in the 5′ half. To determine the evolutionary mechanisms responsible for this pattern, we sequenced a 1612-bp segment of the white gene from an additional 33 lines of D. melanogaster from a European and a North American population. This 1612-bp segment encompasses an 834bp region of the white gene in which the polymorphisms form high frequency haplotypes that cannot be explained by a neutral equilibrium model of molecular evolution. The small number of recombinants in the 834bp region suggests epistatic selection as the cause of the haplotypic structuring, while an investigation of nucleotide diversity supports a directional selection hypothesis. A multi-locus selection model that combines features from both-hypotheses and takes the recent history of D. melanogaster into account may be the best explanation for these data.

scholarly journals SARS-CoV-2 infection and recurrence of anti-glomerular basement disease: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Alexander Winkler ◽  
Emanuel Zitt ◽  
Hannelore Sprenger-Mähr ◽  
Afschin Soleiman ◽  
Manfred Cejna ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Plasma Cells ◽  
Rapidly Progressive Glomerulonephritis ◽  
Kidney Injury ◽  
Pulmonary Involvement ◽  
Pulmonary Haemorrhage ◽  
Basement Membranes ◽  
High Avidity ◽  
History Of

Abstract Background Anti-glomerular basement membrane disease (GBM) disease is a rare autoimmune disease causing rapidly progressive glomerulonephritis and pulmonary haemorrhage. Recently, an association between COVID-19 and anti-glomerular basement membrane (anti-GBM) disease has been proposed. We report on a patient with recurrence of anti-GBM disease after SARS-CoV-2 infection. Case presentation The 31-year-old woman had a past medical history of anti-GBM disease, first diagnosed 11 years ago, and a first relapse 5 years ago. She was admitted with severe dyspnoea, haemoptysis, pulmonary infiltrates and acute on chronic kidney injury. A SARS-CoV-2 PCR was positive with a high cycle threshold. Anti-GBM autoantibodies were undetectable. A kidney biopsy revealed necrotising crescentic glomerulonephritis with linear deposits of IgG, IgM and C3 along the glomerular basement membrane, confirming a recurrence of anti-GBM disease. She was treated with steroids, plasma exchange and two doses of rituximab. Pulmonary disease resolved, but the patient remained dialysis-dependent. We propose that pulmonary involvement of COVID-19 caused exposure of alveolar basement membranes leading to the production of high avidity autoantibodies by long-lived plasma cells, resulting in severe pulmonary renal syndrome. Conclusion Our case supports the assumption of a possible association between COVID-19 and anti-GBM disease.

scholarly journals Tuberculosis presenting as dacryoadenitis in the USA

2019 ◽  
Vol 12 (11) ◽  
pp. e231694 ◽  
Author(s):  
Harry Ross Powers ◽  
Mark Anthony Diaz ◽  
Julio C Mendez
Keyword(s):  
Correct Diagnosis ◽  
Developed Countries ◽  
Histopathological Analysis ◽  
Orbital Mass ◽  
Antituberculosis Therapy ◽  
The Usa ◽  
History Of ◽  
Patient’S History ◽  
High Degree ◽  
Filipino Woman

A 25-year-old Filipino woman living in the USA was evaluated for a 5-month history of left eye pain and a subsequent orbital mass. Histopathological analysis of the lacrimal mass showed a mixed inflammatory process with necrotising granulomas and positive cultures for Mycobacterium tuberculosis. She was treated with antituberculosis therapy, with resolution of symptoms. Tuberculosis dacryoadenitis is extremely rare in the USA and other developed countries. It requires a high degree of clinical suspicion with special attention to the patient’s history to make the correct diagnosis. It can be treated successfully with antituberculosis therapy.

scholarly journals Becker muscular dystrophy associated with sarcomeric hypertrophic cardiomyopathy in a paediatric patient: a case report

2019 ◽  
Vol 3 (3) ◽  
Author(s):  
Paola Dolader ◽  
Ella Field ◽  
Anna Sarkozy ◽  
Juan Pablo Kaski
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Muscular Dystrophy ◽  
Neuromuscular Disorders ◽  
Elevated Serum ◽  
Becker Muscular Dystrophy ◽  
Neuromuscular Disorder ◽  
Chain Gene ◽  
Septal Hypertrophy ◽  
History Of ◽  
Myocardial Involvement

Abstract Background  Becker muscular dystrophy (BMD) is a neuromuscular disorder associated with myocardial involvement. The most frequent presentation is dilated cardiomyopathy. There have been isolated reports of hypertrophic cardiomyopathy (HCM) in association with BMD, but it is unclear whether these patients had an additional aetiology. Case summary  A 10-year-old boy was diagnosed with BMD having presented with a history of muscular pain during exercise and elevated serum creatine kinase levels. A cardiac screening was arranged and the echocardiogram confirmed an asymmetric septal hypertrophy. Given the unusual finding of HCM in this patient with BMD, we performed genetic testing for HCM-causing mutations and identified a likely pathogenic variant in heterozygosis in the beta-myosin heavy chain gene. Discussion  This case highlights the importance of considering additional aetiologies of cardiac disease in the presence of infrequent phenotypic expressions in neuromuscular disorders.

scholarly journals Modelling the COVID-19 pandemic in context: an international participatory approach

2020 ◽  
Vol 5 (12) ◽  
pp. e003126
Author(s):  
Ricardo Aguas ◽  
Lisa White ◽  
Nathaniel Hupert ◽  
Rima Shretta ◽  
Wirichada Pan-Ngum ◽  
...  
Keyword(s):  
Mathematical Models ◽  
Low Income ◽  
Scientific Evidence ◽  
Participatory Approach ◽  
Middle Income ◽  
Middle Income Countries ◽  
Containment Measures ◽  
History Of ◽  
High Degree ◽  
The Mathematical Model

The SARS-CoV-2 pandemic has had an unprecedented impact on multiple levels of society. Not only has the pandemic completely overwhelmed some health systems but it has also changed how scientific evidence is shared and increased the pace at which such evidence is published and consumed, by scientists, policymakers and the wider public. More significantly, the pandemic has created tremendous challenges for decision-makers, who have had to implement highly disruptive containment measures with very little empirical scientific evidence to support their decision-making process. Given this lack of data, predictive mathematical models have played an increasingly prominent role. In high-income countries, there is a long-standing history of established research groups advising policymakers, whereas a general lack of translational capacity has meant that mathematical models frequently remain inaccessible to policymakers in low-income and middle-income countries. Here, we describe a participatory approach to modelling that aims to circumvent this gap. Our approach involved the creation of an international group of infectious disease modellers and other public health experts, which culminated in the establishment of the COVID-19 Modelling (CoMo) Consortium. Here, we describe how the consortium was formed, the way it functions, the mathematical model used and, crucially, the high degree of engagement fostered between CoMo Consortium members and their respective local policymakers and ministries of health.

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