<?xml version="1.0" encoding="UTF-8"?><xml><records><record><source-app name="Biblio" version="7.x">Drupal-Biblio</source-app><ref-type>17</ref-type><contributors><authors><author><style face="normal" font="default" size="100%">Cappelli, Enrico</style></author><author><style face="normal" font="default" size="100%">Cuccarolo, Paola</style></author><author><style face="normal" font="default" size="100%">Stroppiana, Giorgia</style></author><author><style face="normal" font="default" size="100%">Miano, Maurizio</style></author><author><style face="normal" font="default" size="100%">Bottega, Roberta</style></author><author><style face="normal" font="default" size="100%">Cossu, Vanessa</style></author><author><style face="normal" font="default" size="100%">Degan, Paolo</style></author><author><style face="normal" font="default" size="100%">Ravera, Silvia</style></author></authors></contributors><titles><title><style face="normal" font="default" size="100%">Defects in mitochondrial energetic function compels Fanconi Anaemia cells to glycolytic metabolism.</style></title><secondary-title><style face="normal" font="default" size="100%">Biochim Biophys Acta Mol Basis Dis</style></secondary-title><alt-title><style face="normal" font="default" size="100%">Biochim Biophys Acta Mol Basis Dis</style></alt-title></titles><keywords><keyword><style  face="normal" font="default" size="100%">Cell Line</style></keyword><keyword><style  face="normal" font="default" size="100%">Fanconi Anemia</style></keyword><keyword><style  face="normal" font="default" size="100%">Glycolysis</style></keyword><keyword><style  face="normal" font="default" size="100%">Humans</style></keyword><keyword><style  face="normal" font="default" size="100%">Mitochondria</style></keyword><keyword><style  face="normal" font="default" size="100%">Oxidative Phosphorylation</style></keyword><keyword><style  face="normal" font="default" size="100%">Oxidative Stress</style></keyword></keywords><dates><year><style  face="normal" font="default" size="100%">2017</style></year><pub-dates><date><style  face="normal" font="default" size="100%">2017 06</style></date></pub-dates></dates><volume><style face="normal" font="default" size="100%">1863</style></volume><pages><style face="normal" font="default" size="100%">1214-1221</style></pages><language><style face="normal" font="default" size="100%">eng</style></language><abstract><style face="normal" font="default" size="100%">&lt;p&gt;Energetic metabolism plays an essential role in the differentiation of haematopoietic stem cells (HSC). In Fanconi Anaemia (FA), DNA damage is accumulated during HSC differentiation, an event that is likely associated with bone marrow failure (BMF). One of the sources of the DNA damage is altered mitochondrial metabolism and an associated increment of oxidative stress. Recently, altered mitochondrial morphology and a deficit in the energetic activity in FA cells have been reported. Considering that mitochondria are the principal site of aerobic ATP production, we investigated FA metabolism in order to understand what pathways are able to compensate for this energy deficiency. In this work, we report that the impairment in mitochondrial oxidative phosphorylation (OXPHOS) in FA cells is countered by an increase in glycolytic flux. By contrast, glutaminolysis appears lower with respect to controls. Therefore, it is possible to conclude that in FA cells glycolysis represents the main pathway for producing energy, balancing the NADH/NAD ratio by the conversion of pyruvate to lactate. Finally, we show that a forced switch from glycolytic to OXPHOS metabolism increases FA cell oxidative stress. This could be the cause of the impoverishment in bone marrow HSC during exit from the homeostatic quiescent state. This is the first work that systematically explores FA energy metabolism, highlighting its flaws, and discusses the possible relationships between these defects and BMF.&lt;/p&gt;</style></abstract><issue><style face="normal" font="default" size="100%">6</style></issue><custom1><style face="normal" font="default" size="100%">http://www.ncbi.nlm.nih.gov/pubmed/28315453?dopt=Abstract</style></custom1></record><record><source-app name="Biblio" version="7.x">Drupal-Biblio</source-app><ref-type>17</ref-type><contributors><authors><author><style face="normal" font="default" size="100%">Ladogana, Saverio</style></author><author><style face="normal" font="default" size="100%">Maruzzi, Matteo</style></author><author><style face="normal" font="default" size="100%">Samperi, Piera</style></author><author><style face="normal" font="default" size="100%">Perrotta, Silverio</style></author><author><style face="normal" font="default" size="100%">Del Vecchio, Giovanni C</style></author><author><style face="normal" font="default" size="100%">Notarangelo, Lucia D</style></author><author><style face="normal" font="default" size="100%">Farruggia, Piero</style></author><author><style face="normal" font="default" size="100%">Verzegnassi, Federico</style></author><author><style face="normal" font="default" size="100%">Masera, Nicoletta</style></author><author><style face="normal" font="default" size="100%">Saracco, Paola</style></author><author><style face="normal" font="default" size="100%">Fasoli, Silvia</style></author><author><style face="normal" font="default" size="100%">Miano, Maurizio</style></author><author><style face="normal" font="default" size="100%">Girelli, Gabriella</style></author><author><style face="normal" font="default" size="100%">Barcellini, Wilma</style></author><author><style face="normal" font="default" size="100%">Zanella, Alberto</style></author><author><style face="normal" font="default" size="100%">Russo, Giovanna</style></author></authors><translated-authors><author><style face="normal" font="default" size="100%">AIHA Committee of the Italian Association of Paediatric Onco-haematology (AIEOP)</style></author></translated-authors></contributors><titles><title><style face="normal" font="default" size="100%">Diagnosis and management of newly diagnosed childhood autoimmune haemolytic anaemia. Recommendations from the Red Cell Study Group of the Paediatric Haemato-Oncology Italian Association.</style></title><secondary-title><style face="normal" font="default" size="100%">Blood Transfus</style></secondary-title><alt-title><style face="normal" font="default" size="100%">Blood Transfus</style></alt-title></titles><keywords><keyword><style  face="normal" font="default" size="100%">Anemia, Hemolytic, Autoimmune</style></keyword><keyword><style  face="normal" font="default" size="100%">Blood Transfusion</style></keyword><keyword><style  face="normal" font="default" size="100%">Child</style></keyword><keyword><style  face="normal" font="default" size="100%">Coombs Test</style></keyword><keyword><style  face="normal" font="default" size="100%">Disease Management</style></keyword><keyword><style  face="normal" font="default" size="100%">Hematology</style></keyword><keyword><style  face="normal" font="default" size="100%">Humans</style></keyword><keyword><style  face="normal" font="default" size="100%">Immunoglobulin M</style></keyword><keyword><style  face="normal" font="default" size="100%">Italy</style></keyword><keyword><style  face="normal" font="default" size="100%">Pediatrics</style></keyword><keyword><style  face="normal" font="default" size="100%">Societies, Medical</style></keyword><keyword><style  face="normal" font="default" size="100%">Steroids</style></keyword></keywords><dates><year><style  face="normal" font="default" size="100%">2017</style></year><pub-dates><date><style  face="normal" font="default" size="100%">2017 May</style></date></pub-dates></dates><volume><style face="normal" font="default" size="100%">15</style></volume><pages><style face="normal" font="default" size="100%">259-267</style></pages><language><style face="normal" font="default" size="100%">eng</style></language><abstract><style face="normal" font="default" size="100%">&lt;p&gt;Autoimmune haemolytic anaemia is an uncommon disorder to which paediatric haematology centres take a variety of diagnostic and therapeutic approaches. The Red Cell Working Group of the Italian Association of Paediatric Onco-haematology (Associazione Italiana di Ematologia ed Oncologia Pediatrica, AIEOP) developed this document in order to collate expert opinions on the management of newly diagnosed childhood autoimmune haemolytic anaemia.The diagnostic process includes the direct and indirect antiglobulin tests; recommendations are given regarding further diagnostic tests, specifically in the cases that the direct and indirect antiglobulin tests are negative. Clear-cut definitions of clinical response are stated. Specific recommendations for treatment include: dosage of steroid therapy and tapering modality for warm autoimmune haemolytic anaemia; the choice of rituximab as first-line therapy for the rare primary transfusion-dependent cold autoimmune haemolytic anaemia; the indications for supportive therapy; the need for switching to second-line therapy. Each statement is provided with a score expressing the level of appropriateness and the agreement among participants.&lt;/p&gt;</style></abstract><issue><style face="normal" font="default" size="100%">3</style></issue><custom1><style face="normal" font="default" size="100%">http://www.ncbi.nlm.nih.gov/pubmed/28151390?dopt=Abstract</style></custom1></record></records></xml>