scholarly journals Unraveling the Mystery of Canavan Disease: A 100 Year Chronicle of Key Events

2019 ◽  
Vol 1 (2) ◽  
Keyword(s):  
Muscle Tone ◽  
Canavan Disease ◽  
Short Review ◽  
Head Movements ◽  
Past Century ◽  
Great Expectations ◽  
The Past ◽  
Pain And Suffering ◽  
Key Events ◽  
Northeastern Europe

In the early 1900’s, in a small geographical region in northeastern Europe, a mysterious early-onset human malady was brought to the attention of physicians. In this disease, children appeared normal at birth and met usual developmental milestones up to the age of about 4-5 months. At that time, infants began to lose muscle tone and could no longer control their head movements. The disease was progressive, and by age 2, characterized by enlargement of the head, spastic responses to stimulation, and a general listlessness. By age 3, blindness was apparent and there was extension and posturing of limbs due to uncontrolled muscle contractions. Death usually occurred between ages 3-4. In large family’s common at that time, there could be 2-3 afflicted children out of a total of 8-9 siblings. There is no metric to measure pain and suffering of CD children, but we can measure progress made over the past century in solving the mystery. At the present time, there are great expectations that a human cure is possible since a mouse model of CD was recently shown to be completely cured by an unusual genetic engineering outcome. In this short review are chronicled key findings made over the past 100 + years that have led to identification of the genetic and cellular etiology of CD, and the reasons for such encouragement.

scholarly journals IAPSO: tales from the ocean frontier

2019 ◽  
Vol 10 (1) ◽  
pp. 137-150
Author(s):  
Denise Smythe-Wright ◽  
W. John Gould ◽  
Trevor J. McDougall ◽  
Stefania Sparnocchia ◽  
Philip L. Woodworth
Keyword(s):  
International Association ◽  
Present Knowledge ◽  
Past Century ◽  
Physical Sciences ◽  
Future Health ◽  
The Past ◽  
Intractable Problems ◽  
Long Time ◽  
Earth’S Climate ◽  
Key Events

Abstract. Our 21st century perspective on the oceans is due to the realization that knowledge of them and specifically their role in earth's climate are central to determining the future health of our planet. This present knowledge of the oceans builds on the farsighted work of people who, over the past century, worked to address seemingly intractable problems. The International Association for the Physical Sciences of the Oceans (IAPSO) has, over that long time span, promoted and supported the international approach that is now commonplace and has championed the provision of cross-cutting activities, the value of which we now fully recognize. This paper describes the key events in IAPSO's history and the roles played by the scientists involved.

The zoonotic potential of bat-borne coronaviruses

2020 ◽  
Vol 4 (4) ◽  
pp. 365-381
Author(s):  
Ny Anjara Fifi Ravelomanantsoa ◽  
Sarah Guth ◽  
Angelo Andrianiaina ◽  
Santino Andry ◽  
Anecia Gentles ◽  
...  
Keyword(s):  
Genetic Material ◽  
Field Research ◽  
Sympatric Species ◽  
Animal Origin ◽  
Mammalian Host ◽  
Past Century ◽  
The Past ◽  
Novel Host ◽  
Horseshoe Bat ◽  
Human Infections

Seven zoonoses — human infections of animal origin — have emerged from the Coronaviridae family in the past century, including three viruses responsible for significant human mortality (SARS-CoV, MERS-CoV, and SARS-CoV-2) in the past twenty years alone. These three viruses, in addition to two older CoV zoonoses (HCoV-229E and HCoV-NL63) are believed to be originally derived from wild bat reservoir species. We review the molecular biology of the bat-derived Alpha- and Betacoronavirus genera, highlighting features that contribute to their potential for cross-species emergence, including the use of well-conserved mammalian host cell machinery for cell entry and a unique capacity for adaptation to novel host environments after host switching. The adaptive capacity of coronaviruses largely results from their large genomes, which reduce the risk of deleterious mutational errors and facilitate range-expanding recombination events by offering heightened redundancy in essential genetic material. Large CoV genomes are made possible by the unique proofreading capacity encoded for their RNA-dependent polymerase. We find that bat-borne SARS-related coronaviruses in the subgenus Sarbecovirus, the source clade for SARS-CoV and SARS-CoV-2, present a particularly poignant pandemic threat, due to the extraordinary viral genetic diversity represented among several sympatric species of their horseshoe bat hosts. To date, Sarbecovirus surveillance has been almost entirely restricted to China. More vigorous field research efforts tracking the circulation of Sarbecoviruses specifically and Betacoronaviruses more generally is needed across a broader global range if we are to avoid future repeats of the COVID-19 pandemic.

PCSK9 inhibition for LDL lowering and beyond – implications for patients with peripheral artery disease

VASA ◽  
2018 ◽  
Vol 47 (3) ◽  
pp. 165-176 ◽  
Author(s):  
Katrin Gebauer ◽  
Holger Reinecke
Keyword(s):  
Cardiovascular Risk ◽  
Risk Reduction ◽  
Ldl Receptor ◽  
Density Lipoprotein ◽  
Past Century ◽  
Pcsk9 Inhibitors ◽  
Cardiovascular Risk Reduction ◽  
Simultaneous Application ◽  
The Past ◽  
Pcsk9 Inhibition

Abstract. Low-density lipoprotein cholesterol (LDL-C) has been proven to be a causal factor of atherosclerosis and, along with other triggers like inflammation, the most frequent reason for peripheral arterial disease. Moreover, a linear correlation between LDL-C concentration and cardiovascular outcome in high-risk patients could be established during the past century. After the development of statins, numerous randomized trials have shown the superiority for LDL-C reduction and hence the decrease in cardiovascular outcomes including mortality. Over the past decades it became evident that more intense LDL-C lowering, by either the use of highly potent statin supplements or by additional cholesterol absorption inhibitor application, accounted for an even more profound cardiovascular risk reduction. Proprotein convertase subtilisin/kexin type 9 (PCSK9), a serin protease with effect on the LDL receptor cycle leading to its degradation and therefore preventing continuing LDL-C clearance from the blood, is the target of a newly developed monoclonal antibody facilitating astounding LDL-C reduction far below to what has been set as target level by recent ESC/EAS guidelines in management of dyslipidaemias. Large randomized outcome trials including subjects with PAD so far have been able to prove significant and even more intense cardiovascular risk reduction via further LDL-C debasement on top of high-intensity statin medication. Another approach for LDL-C reduction is a silencing interfering RNA muting the translation of PCSK9 intracellularly. Moreover, PCSK9 concentrations are elevated in cells involved in plaque composition, so the potency of intracellular PCSK9 inhibition and therefore prevention or reversal of plaques may provide this mechanism of action on PCSK9 with additional beneficial effects on cells involved in plaque formation. Thus, simultaneous application of statins and PCSK9 inhibitors promise to reduce cardiovascular event burden by both LDL-C reduction and pleiotropic effects of both agents.

Archaeology in the Past Century

1901 ◽  
Vol 51 (1309supp) ◽  
pp. 20976-20977
Author(s):  
W. M. Flinders Petrje
Keyword(s):  
Past Century ◽  
The Past

Catholicism and Cosmopolitanism: the Confluence of Three Catholic Scholars and the Cosmopolitan Democrats on State Sovereignty and the Future of Global Governance

2016 ◽  
Vol 3 (2) ◽  
pp. 37-51
Author(s):  
Matthew Bagot
Keyword(s):  
Global Governance ◽  
State Sovereignty ◽  
Current Debate ◽  
Past Century ◽  
Jacques Maritain ◽  
The Past ◽  
Technological Advances ◽  
The Future ◽  
Cosmopolitan Democracy ◽  
Catholic Tradition

One of the central questions in international relations today is how we should conceive of state sovereignty. The notion of sovereignty—’supreme authority within a territory’, as Daniel Philpott defines it—emerged after the Treaty of Westphalia in 1648 as a result of which the late medieval crisis of pluralism was settled. But recent changes in the international order, such as technological advances that have spurred globalization and the emerging norm of the Responsibility to Protect, have cast the notion of sovereignty into an unclear light. The purpose of this paper is to contribute to the current debate regarding sovereignty by exploring two schools of thought on the matter: first, three Catholic scholars from the past century—Luigi Sturzo, Jacques Maritain, and John Courtney Murray, S.J.—taken as representative of Catholic tradition; second, a number of contemporary political theorists of cosmopolitan democracy. The paper argues that there is a confluence between the Catholic thinkers and the cosmopolitan democrats regarding their understanding of state sovereignty and that, taken together, the two schools have much to contribute not only to our current understanding of sovereignty, but also to the future of global governance.

Aftershocks

2017 ◽  
Author(s):  
Seva Gunitsky
Keyword(s):  
Twentieth Century ◽  
Regime Change ◽  
International System ◽  
Political Legitimacy ◽  
Great Powers ◽  
Past Century ◽  
The Past ◽  
Sudden Rise ◽  
The World ◽  
National Borders

Over the past century, democracy spread around the world in turbulent bursts of change, sweeping across national borders in dramatic cascades of revolution and reform. This book offers a new global-oriented explanation for this wavelike spread and retreat—not only of democracy but also of its twentieth-century rivals, fascism, and communism. The book argues that waves of regime change are driven by the aftermath of cataclysmic disruptions to the international system. These hegemonic shocks, marked by the sudden rise and fall of great powers, have been essential and often-neglected drivers of domestic transformations. Though rare and fleeting, they not only repeatedly alter the global hierarchy of powerful states but also create unique and powerful opportunities for sweeping national reforms—by triggering military impositions, swiftly changing the incentives of domestic actors, or transforming the basis of political legitimacy itself. As a result, the evolution of modern regimes cannot be fully understood without examining the consequences of clashes between great powers, which repeatedly—and often unsuccessfully—sought to cajole, inspire, and intimidate other states into joining their camps.

Variable oil and argumentative conflict in the Middle East

2020 ◽  
pp. 824
Author(s):  
Malik Daham Mata’ab
Keyword(s):  
Middle East ◽  
Past Century ◽  
Large Area ◽  
The Past ◽  
The World

Oil has formed since its discovery so far one of the main causes of global conflict, has occupied this energy map a large area of conflict the world over the past century, and certainly this matter will continue for the next period in our century..

Mahmood Hasan Khan. Underdevelopment and Agrarian Structure in Pakistan. Boulder, Colorado: Westview Press. 1981. pp.xviii+ 307.

1981 ◽  
Vol 20 (4) ◽  
pp. 465-468 ◽  
Author(s):  
Douglas H. Perry
Keyword(s):  
Ownership Structure ◽  
Land Reform ◽  
Land Ownership ◽  
Past Century ◽  
The Past ◽  
Ownership Patterns ◽  
Agrarian Structure

Students of the land ownership patterns in Pakistan have always been hampered by extreme lack of data, neither the 1960 census nor the 1972 census reveal anything about the actual ownership structure of land. Khan's book goes some distance in providing numbers on land ownership (for 1971 and 1976), and also documents methods and failures of land reform efforts over the past century in Pakistan, disaggregated to show efforts in this regard in both the provinces of Sind and Punjab. The book actually provides an overwhelming amount of data - some 87 pages of charts and tables document a book of under 200 pages of text.

Great Expectations

2008 ◽  
Author(s):  
Charles Dickens ◽  
Robert Douglas-Fairhurst
Keyword(s):  
Great Expectations ◽  
The Past ◽  
Theatrical Adaptation

‘You are to understand, Mr. Pip, that the name of the person who is your liberal benefactor remains a profound secret.’ Young Pip lives with his sister and her husband the blacksmith, with few prospects for advancement until a mysterious benefaction takes him from the Kent marshes to London. Pip is haunted by figures from his past - the escaped convict Magwitch, the time-withered Miss Havisham and her proud and beautiful ward, Estella - and in time uncovers not just the origins of his great expectations but the mystery of his own heart. A powerful and moving novel, Great Expectations is suffused with Dickens’s memories of the past and its grip on the present, and it raises disturbing questions about the extent to which individuals affect each other’s lives. This edition includes a lively introduction, Dickens’s working notes, the novel’s original ending, and an extract from an early theatrical adaptation. It reprints the definitive Clarendon text.

The Future of FBI Counterintelligence through the Lens of the Past Hundred Years

2010 ◽  
pp. 504-517
Author(s):  
Raymond J. Batvinis
Keyword(s):  
United States ◽  
The United States ◽  
Main Concern ◽  
Federal Bureau ◽  
The Past ◽  
Intelligence Services ◽  
Terrorist Groups ◽  
Foreign Intelligence ◽  
Key Events ◽  
The U.S

Counterintelligence is the business of identifying and dealing with foreign intelligence threats to a nation, such as the United States. Its main concern is the intelligence services of foreign states and similar organizations of non-state actors, such as transnational terrorist groups. Counterintelligence functions both as a defensive measure that protects the nation's secrets and assets against foreign intelligence penetration and as an offensive measure to find out what foreign intelligence organizations are planning to defeat better their aim. This article addresses the Federal Bureau of Investigation's (FBI) foreign counterintelligence function. It briefly traces its evolution by examining the key events and the issues that effected its growth as the principle civilian counterintelligence service of the U.S. government.

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