“
“Neuronal ceroid lipofuscinoses (NCLs) are pediatric, neurodegenerative, lysosomal storage disorders. Mutations in cathepsin D result in the most severe, Captisol congenital form of NCLs. We have previously generated a cathepsin D deficient Drosophila model, which exhibits the key features of NCLs: progressive intracellular accumulation of autofluorescent storage material and modest neurodegeneration in the brain areas related to visual functions. Here we extend the phenotypic characterization of cathepsin D deficient

Drosophila and report that modest degenerative changes are also present in their retinae. Furthermore, by utilizing this phenotype, we examined the possible effect of 17 candidate modifiers, selected based on the results from other cathepsin D deficiency models. We found enhancers of this phenotype that support the involvement of endocytosis-, lipid metabolism- and oxidation-related factors in the cathepsin D deficiency induced degeneration. Our results warrant further investigation of these mechanisms

in the pathogenesis of cathepsin D deficiency. (C) 2009 Elsevier Inc. All rights reserved.”
“Purpose: To evaluate positron emission tomography computed tomography (PET-CT) influences in involved-field radiation therapy (IFRT) field design PF-02341066 Protein Tyrosine Kinase inhibitor in pediatric Hodgkin’s lymphoma (HL).\n\nMaterials and Methods: From June 2003 to February 2008, 30 pediatric HL patients were treated at Children’s Healthcare of Atlanta (CHOA) and Emory University Department of Radiation Oncology with both chemotherapy and IFRT. Diagnostic contrast-enhanced CT and PET-CT were coregistered using image fusion software. Both were

reviewed for all potential sites of involvement and correlated buy BIIB057 to determine concordance and discordance. They were used in IFRT planning to determine the influence of PET-CT on target volumes and field design.\n\nResults: There were 546 regions analyzed by both PET and CT modalities. Image sets were concordant in 468 regions and discordant in 78, yielding 86% concordance overall. Analysis by weighted K statistic showed “intermediate to good” fit overall and for nodal sites, but “poor” agreement for extranodal sites. If discordant, a site was most likely PET+/CT-. Integration of PET information caused a change in staging in 15(50%) patients, 7 upstaged and 8 downstaged. The IFRT volumes were adjusted on the basis of initial PET-CT finding in 21 (70%) patients, with 32 sites added and 15 excluded. There were four relapses, only one outside IFRT fields, but all were successfully salvaged.\n\nConclusion: PET-CT represents an important tool in the management of pediatric patients with HL and has a substantial influence on both initial staging and radiation treatment target definition and field design. (C) 2011 Elsevier Inc.”
“Background: Bupleurum chinense DC. is a widely used traditional Chinese medicinal plant. Saikosaponins are the major bioactive constituents of B.