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In This Issue
Role of infection in urea cycle disorders
Disease Models & Mechanisms 2014 7: e205
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Ornithine transcarbamylase deficiency (OTCD) is a type of urea cycle disorder – a disease in which the body fails to clear toxic ammonia from the bloodstream. The accumulation of ammonia in the blood (hyperammonaemia) can lead to potentially life-threatening metabolic disturbances in affected individuals, known as acute metabolic decompensation. Several factors, including infection, are thought to precipitate these metabolic aberrations. In this study, Peter McGuire and colleagues identify infection as the most common cause of acute decompensation in a prospective cohort of individuals with OTCD. To explore the metabolic changes that occur, they developed an experimental mouse model in which decompensation with hyperammonaemia is triggered by influenza infection. In response to infection, mice demonstrated altered hepatic immune function and reductions in urea cycle enzyme activity and urea cycle intermediates. These findings could help guide the development of new approaches to manage acute metabolic decompensation in OTCD and related inborn errors of metabolism. Page 205

  • © 2014. Published by The Company of Biologists Ltd

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.

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Role of infection in urea cycle disorders
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DMM and COVID-19

We are aware that the COVID-19 pandemic is having an unprecedented impact on researchers worldwide. The Editors of all The Company of Biologists’ journals have been considering ways in which we can alleviate concerns that members of our community may have around publishing activities during this time. Read about the actions we are taking at this time.

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Professor Elizabeth Patton appointed as DMM’s next Editor-in-Chief

We are pleased to announce that The Company of Biologists directors have appointed Professor Elizabeth Patton as DMM's new Editor-in-Chief. As Paresh Vyas writes in his Editorial, Liz ‘brings vitality and a passion for the remit of DMM, and is deeply embedded in the community.’


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Identification of MYOM2 as a candidate gene in hypertrophic cardiomyopathy and Tetralogy of Fallot, and its functional evaluation in the Drosophila heart

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Interview – Kim Landry-Truchon and Nicolas Houde

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