Larval dietary protein complexity affects the regulation of muscle growth and the expression of DNA methyltransferases in Senegalese sole

Due to its high protein synthesis and deposition rates, skeletal muscle protein deposition is a major determinant of fish growth. Dietary protein complexity is likely to influence protein utilization and deposition in skeletal muscle, possibly affecting fish myogenesis. In this study, three microdiets were formulated with different degree of hydrolysis of dietary protein as the changing factor: one diet contained a mix of intact protein sources targeting a peptide with molecular weight >20 kDa (Intact); a second diet contained a hydrolysate with polypeptides ranging from 5 to 70 kDa (PartH); and a third diet contained a high level of a protein hydrolysate mostly composed of small peptides (<5 kDa) (HighH). A possible effect on the regulation of muscle growth in Senegalese sole larvae was evaluated through white muscle cellularity and the expression of muscle growth-related genes at 16 and 36 DAH. The PartH diet promoted white muscle growth during the metamorphosis climax (16 DAH), which was reflected on increased body weight. At 36 DAH, different diets induced different expression patterns of genes encoding for the myogenic regulatory factors, which affected muscle growth dynamics, ultimately promoting growth potential in the Intact group. A lower recruitment of small-sized fibres in the PartH and HighH groups led to reduced potential for muscle growth, which resulted on further reduced somatic growth. Accordingly, fish fed the Intact diet grew better up to a late juvenile stage (60 DAH) and were still heavier than others even after 30 days of feeding all groups on the same commercial diet, at 90 DAH. The up-regulation in the transcript levels of genes encoding for de novo DNA methyltransferases in the HighH group suggest a potential for nutritional programming in this species.This work was funded by the Project EPISOLE (FCT) [PTDC/MAR/110547/2009], through project CCMAR/Multi/04326/2013 (Portugal) from FCT (Portugal), and by the project MICALA — I&DT Co-Promoção No. 13380 (Portugal, supported by POAlgarve 21, QREN and European Union). P. Canada was supported by FCT grant SFRH/BD/82149/2011. Sofia Engrola was supported by FCT investigator grant IF/00482/2014/CP1217/CT0005 funded by the European Social Fund, the Operational Programme Human Potential and the Foundation for Science and Technology of Portugal (FCT).Peer Reviewe

ADPedKD: A Global Online Platform on the Management of Children With ADPKD

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization. Methods: Global ADPedKD is an international multicenter observational study focusing on childhood-diagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions. Discussion: The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease

ADPedKD: A Global Online Platform on the Management of Children With ADPKD

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization. Methods: Global ADPedKD is an international multicenter observational study focusing on childhooddiagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions. Discussion: The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease