PDGF-BB serum levels are decreased in adult onset Pompe patients

Adult onset Pompe disease is a genetic disorder characterized by slowly progressive skeletal and respiratory muscle weakness. Symptomatic patients are treated with enzymatic replacement therapy with human recombinant alfa glucosidase. Motor functional tests and spirometry are commonly used to follow patients up. However, a serological biomarker that correlates with the progression of the disease could improve follow-up. We studied serum concentrations of TGFβ, PDGF-BB, PDGF-AA and CTGF growth factors in 37 adult onset Pompe patients and 45 controls. Moreover, all patients performed several muscle function tests, conventional spirometry, and quantitative muscle MRI using 3-point Dixon. We observed a statistically significant change in the serum concentration of each growth factor in patients compared to controls. However, only PDGF-BB levels were able to differentiate between asymptomatic and symptomatic patients, suggesting its potential role in the follow-up of asymptomatic patients. Moreover, our results point to a dysregulation of muscle regeneration as an additional pathomechanism of Pompe disease

Longitudinal data assessment of global stability index in kale leaves

ABSTRACT Kale plants are usually sold “in natura” in street markets and malls. Kale leaves can have their appearance compromised by dehydration and discoloration due to increased post-harvest time exposure. We aimed to analyze the Global Stability Index (GSI) in kale accessions by means of repeated measurement analysis and curve grouping as a complementary form of superior sample identification with regard to post-harvest preservation. Thirty kale accessions were evaluated using a randomized block design with four blocks and five plants per plot. Two commercial leaves per plant were collected, and kept on workbenches in the shade at a temperature of 18 ± 1 °C. Subsequently, the degrees of discoloration and dehydration, total chlorophyll content, and accumulated fresh mass loss were evaluated over a 15-day period. From these data, the GSI was calculated for each day of evaluation. In addition, using mixed models, thirteen co-variance structures were tested. For graphical analysis, thirteen linear and non-linear models were assessed followed by curve grouping using multivariate analysis. The GSI was efficient for differentiating accessions, which became an important tool in post-harvest studies. GSI values were not equally correlated, therefore the use of mixed models became an important approach. The unstructured matrix was the best fit to model the dependence of error. The Melow I model was the best fit for studying the GSI. The accessions UFVJM-10, UFLA-1, COM-1, UFVJM-32, COM-3, UFVJM-8, UFVJM-36 and UFVJM-24, belonging to 3 and 5 clusters, are recommended for crop cultivation and as parental material in breeding programs