BACKGROUND Cells respond to stress stimuli through a number of response pathways, of which one of the most important and well characterized is the unfolded protein response. Despite a large body of work which suggests that stress in erythroblasts may play a pivotal role in the pathogenesis of beta-thalassemia/Hb E disease, this pathway remains uninvestigated. DESIGN AND METHODS Day 10 erythro...

MJ Fonseca, A Moreira, P Moreira, L Delgado, V Teixeira, P Padrão Faculty of Nutrition and Food Sciences, University of Porto, Porto, Portugal Department of Immunology, Faculty of Medicine, University of Porto, Porto, Portugal Department of Immunoallergology, Hospital of São João, Porto, Porto, Portugal Research Centre in Physical Activity, Health and Leisure, University of Porto, Porto, Portug...

Besides sickle-cell anaemia, the finding of Hb S with other /3-chain variants of adult haemoglobin was first demonstrated by Itano and Neel (1950) in the well-known discovery of sicklecell Hb C disease. Since then Hb S has been observed with Hb D, E, G, J. and K (Itano, 1951; Aksoy and Lehmann, 1957; Schwartz and Spaet, 1955 ; Went and MacIver, 1959; O'Gorman et al., 1963). In such circumstance...

Materials 9 patients with CAPS (6 CINCA and 3 Muckle-Wells syndromesMWS) were included in our study. In all patients genetic tests in CIAS1 gene was performed. 4 patients have positive mutations (3 CINCA and 1 MWS) and 5 have not any mutations in CIAS1 gene (3 CINCA and 2 MWS). Disease activity was measured with applying simplified auto-inflammatory disease activity index (sAIDAI, M.Piram et al...

In this study, hyperbranched polymers were explored as matrix modifiers to create E-glass fiber (GF) reinforced polymer composites with enhanced mechanical properties. Hyperbranched have lower viscosities than their linear equivalents, potentially providing wet out leading improved stress transfer. (HB), hydrogenated (H-HB), and functional (LF) divinyl benzene blended polystyrene (LP) form a ra...

The phenotypic and molecular diversity of compound heterozygosity for thalassemic genes in Greece is extensive. The interaction of silent and classic β-thalassemia results in the clinical phenotype of thalassemia intermedia. We report the clinical and hematological findings in an 18-year-old female referred to our hemoglobinopathy prevention unit that was observed to be compound heterozygote fo...

Authors' A Oncology, Netherland Medicine Pharmaceu Correspon icine, Radb HB Nijmeg 3618942. E