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- The use of prealbumin concentration as a biomarker of nutritional status in treated phenylketonuric patientsPublication . Rocha, Júlio César; Almeida, Manuela Ferreira; Carmona, Carla; Cardoso, Maria Luís; Borges, Nuno; Soares, Isabel; Salcedo, Graça; Lima, Margarida Reis; Azevedo, Isabel; Van Spronsen, Francjan J.BACKGROUND/AIMS: The neurological sequelae resulting from untreated phenylketonuria are diminished by the success of early introduced and continued dietary treatment. Nowadays, nutritional status is gaining importance in the follow-up of these patients. The aim of this work was to study the relevance of prealbumin concentration as biomarker of protein nutritional status of phenylketonuric patients. METHODS: We collected data from 69 phenylketonuric patients on food intake, blood prealbumin and blood phenylalanine concentrations. Protein insufficiency was defined as prealbumin z-scores below the 5th percentile of reference population. Additionally, we considered a prealbumin concentration of 20 mg/dl as a threshold level. RESULTS: Nine patients (13%) showed signs of protein insufficiency. When the threshold of 20 mg/dl for prealbumin was used, we found 38 patients (55%) with low prealbumin concentrations. CONCLUSION: A significant group presented signs of protein insufficiency either using prealbumin z-scores or prealbumin concentration threshold, especially in milder forms of the disease. The results of this seem to confirm the already described threshold level for prealbumin concentration, suggesting that its measurement may be important for nutritional status evaluation, preventing protein insufficiency in milder forms of phenylketonuria.
- Rescue of F508del-CFTR by RXR motif inactivation triggers proteome modulation associated with the unfolded protein responsePublication . Gomes-Alves, Patrícia; Couto, Francisco; Pesquita, Cátia; Coelho, Ana V.; Penque, DeborahF508del-CFTR, the most common mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, disrupts intracellular trafficking leading to cystic fibrosis (CF). The trafficking defect of F508del-CFTR can be rescued by simultaneous inactivation of its four RXR motifs (4RK). Proteins involved in the F508del-CFTR trafficking defect and/or rescue are therefore potential CF therapeutic targets. We sought to identify these proteins by investigating differential proteome modulation in BHK cells over-expressing wt-CFTR, F508del-CFTR or the revertant F508del/4RK-CFTR. By 2-dimensional electrophoresis-based proteomics and western blot approaches we demonstrated that over-expression of F508del/4RK-CFTR modulates the expression of a large number of proteins, many of which are reported interactors of CFTR and/ or 14-3-3 with potential roles in CFTR trafficking. GRP78/BiP, a marker of ER stress and unfolded protein response (UPR), is up-regulated in cells over-expressing either F508del-CFTR or F598del/4RK-CFTR. However, over-expression of F508del/4RK-CFTR induces the up-regulation of many other UPR-associated proteins (e.g. GRP94, PDI, GRP75/mortalin) and, interestingly, the down-regulation of proteasome components associated with CFTR degradation, such as the proteasome activator PA28 (PSME2) and COP9 signalosome (COPS5/CSN5). Moreover, the F508del-CFTR-induced proteostasis imbalance, which involves some heat shock chaperones (e.g. HSP72/Hpa2), ER-EF-hand Ca2+-binding proteins (calumenin) and the proteasome activator PA28 (PSME2), tends to be ‘restored’, i.e., in BHK cells over-expressing F508del/4RK-CFTR those proteins tend to have expression levels similar to the wild-type ones. These findings indicate that a particular cellular environment orchestrated by the UPR contributes to and/or is compatible with F508del/4RK-CFTR rescue.
- Relatório REVIVE 2008/2009: Programa Nacional de Vigilância de Vectores CulicídeosPublication . Centro de Estudos de Vetores e Doenças Infecciosas Doutor Francisco Cambournac; Alves, Maria João; Osório, Hugo; Zé-Zé, Líbia; Amaro, Fátima
- Outbreak of acute respiratory infection among infants in Lisbon, Portugal, caused by human adenovirus serotype 3 and a new 7/3 recombinant strainPublication . Andrade, Helena Rebelo de; Pereira, Cristiana; Gíria, Marta; Prudêncio, Ema; Brito, Maria João; Calé, Etelvina; Taveira, NunoHuman adenoviruses (AdVs) typically cause mild illnesses in otherwise healthy hosts. We investigated a pediatric outbreak of acute respiratory infection with fatal outcomes that occurred in Lisbon, Portugal, in 2004. Biological specimens were collected from 83 children attending two nurseries, a kinesiotherapy clinic, and the household of a nanny. Adenovirus infection was confirmed in 48 children by PCR and virus isolation. Most (96%) isolates were classified as being of subspecies B1. Phylogenetic analysis of fiber and hexon gene sequences revealed that most infants were infected with AdV serotype 3 (AdV3) strains. Infants attending one nursery harbored a new recombinant strain containing an AdV serotype 7 hexon and serotype 3 fiber (AdV7/3). Both the AdV3 and the AdV7/3 strains caused fatal infections. Two different serotype 3 strains were circulating in Lisbon in 2004, and the new AdV7/3 recombinant type originated from only one of those strains. These results demonstrate that recombination leads to the emergence of new adenovirus strains with epidemic and lethal potential.
- Emerging roles for WNK kinases in cancerPublication . Moniz, Sónia; Jordan, PeterThe subfamily of WNK protein kinases is composed of four human genes and is characterised by a typical sequence variation within the conserved catalytic domain. Although most research has focussed on the role of WNK1, WNK3 and WNK4 in regulating different ion transporters in both the kidney and extrarenal tissues, there is growing evidence for additional roles of WNK kinases in various signalling cascades related to cancer. Here, we review the connection between WNK kinases and tumorigenesis and describe existing experimental evidence as well as potential new links to major aspects of tumour biology. In particular, we discuss their role in G1/S cell cycle progression, metabolic tumour cell adaptation, evasion of apoptosis and metastasis.
- Outbreak of acute respiratory infection among infants in Lisbon, Portugal, caused by human adenovirus serotype 3 and a new 7/3 recombinant strainPublication . Rebelo-de-Andrade, Helena; Pereia, C.; Giria, M.; Prudêncio, E.; Brito, M.J.; Calé, E.; Taveira, N.Human adenoviruses (AdVs) typically cause mild illnesses in otherwise healthy hosts. We investigated a pediatric outbreak of acute respiratory infection with fatal outcomes that occurred in Lisbon, Portugal, in 2004.Biological specimens were collected from 83 children attending two nurseries, a kinesiotherapy clinic, and the household of a nanny. Adenovirus infection was confirmed in 48 children by PCR and virus isolation. Most(96%) isolates were classified as being of subspecies B1. Phylogenetic analysis of fiber and hexon gene sequences revealed that most infants were infected with AdV serotype 3 (AdV3) strains. Infants attending one nursery harbored a new recombinant strain containing an AdV serotype 7 hexon and serotype 3 fiber (AdV7/3). Both the AdV3 and the AdV7/3 strains caused fatal infections. Two different serotype 3 strains were circulating in Lisbon in 2004, and the new AdV7/3 recombinant type originated from only one of those strains. These results demonstrate that recombination leads to the emergence of new adenovirus strains with epidemic and lethal potential
- Proteomic mining of the red blood cell: focus on the membrane proteomePublication . Alexandre, Bruno M.
- Study of the diffusion coefficients of diphenylbutadiene and triclosan into and within meatPublication . Silva, A. Sanches; Freire, J. M. Cruz; Losada, P. PaseiroThe migration of two chemicals, diphenylbutadiene and triclosan, was the target of this paper. Pork meat with different fat contents was prepared to study the influence of this parameter in the migration levels and to study the rate of diffusion of these migrants into the whole plastic/foodstuffs system and within the foodstuff. The whole system plastic/foodstuff diffusion coefficient (effective D) was calculated according to an equation based on the Fick’s Second Law, and D within the foodstuff (DF) was calculated according to the Moisan equation. At 5 C, DF was 1.6 9 10-7 and 1.7 9 10-7 cm2/s for DPBD and triclosan, respectively. At 25 C, DF was 3.7 9 10-7 and 3.9 9 10-7 cm2/s for DPBD and triclosan, respectively. As expected, D within the pork meat is faster than the whole system D, which means that the interface plastic/foodstuff may be the limiting step for the mass transport of chemicals from the packaging to the foodstuff.
- The new automated daily mortality surveillance system in PortugalPublication . Nogueira, P.J.; Machado, A.; Rodrigues, E.; Nunes, Baltazar; Sousa, L.; Jacinto, M.; Ferreira, A.; Falcão, J.M.; Ferrinho, P.The experience reported in an earlier Eurosurveillance issue on a fast method to evaluate the impact of the 2003 heatwave on mortality in Portugal, generated a daily mortality surveillance system (VDM) that has been operating ever since jointly with the Portuguese Heat Health Watch Warning System. This work describes the VDM system and how it evolved to become an automated system operating year-round, and shows briefly its potential using mortality data from January 2006 to June 2009 collected by the system itself. The new system has important advantages such as: rapid information acquisition, completeness (the entire population is included), lightness (very little information is exchanged, date of death, age, sex, place of death registration). It allows rapid detection of impacts (within five days) and allows a quick preliminary quantification of impacts that usually took several years to be done. These characteristics make this system a powerful tool for public health action. The VDM system also represents an example of inter-institutional cooperation, bringing together organisations from two different ministries, Health and Justice, aiming at improving knowledge about the mortality in the population.
- Diagnostic and prognostic biomarker discovery strategies for autoimmune disordersPublication . Gibson, David S.; Banha, João; Penque, Deborah; Costa, Luciana; Conrads, Thomas P.; Cahill, Dolores J.; O'Brien, John K.; Rooney, Madeleine E.Current clinical, laboratory or radiological parameters cannot accurately diagnose or predict disease outcomes in a range of autoimmune disorders. Biomarkers which can diagnose at an earlier time point, predict outcome or help guide therapeutic strategies in autoimmune diseases could improve clinical management of this broad group of debilitating disorders. Additionally, there is a growing need for a deeper understanding of multi-factorial autoimmune disorders. Proteomic platforms offering a multiplex approach are more likely to reflect the complexity of autoimmune disease processes. Findings from proteomic based studies of three distinct autoimmune diseases are presented and strategies compared. It is the authors' view that such approaches are likely to be fruitful in the movement of autoimmune disease treatment away from reactive decisions and towards a preventative stand point.