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Type B

Jitka Rybová, Ladislav Kuchar, Helena Hulková, Befekadu Asfaw, Robert Dobrovolný, Jakub Sikora, Vladimír Havlícek, Ludovít Škultéty, Jana Ledvinová
Blood group B glycosphingolipids (B-GSLs) are substrates of the lysosomal alpha-galactosidase A (AGAL). Similar to its major substrate - globotriaosylceramide (Gb3Cer) - B-GSLs are not degraded and accumulate in the cells of patients affected by an inherited defect of AGAL activity (Fabry disease - FD).The pancreas is a secretory organ known to have high biosynthesis of blood group GSLs. Herein, we provide a comprehensive overview of the biochemical and structural abnormalities in pancreatic tissue from two male FD patients with blood group B...
March 14, 2018: Glycobiology
Francesca Micoli, Paolo Costantino, Roberto Adamo
Cell surface carbohydrates have been proven optimal targets for vaccine development. Conjugation of polysaccharides to a carrier protein triggers a T-cell dependent immune response to the glycan moiety. Licensed glycoconjugate vaccines are produced by chemical conjugation of capsular polysaccharides to prevent meningitis caused by meningococcus, pneumococcus and Haemophilus influenzae type b. However, other classes of carbohydrates (O-antigens, exopolysaccharides, wall/teichoic acids) represent attractive targets for developing vaccines...
March 14, 2018: FEMS Microbiology Reviews
Ji-Eun Kang, Hyun-Do Kim, Soo-Young Park, Jae-Gu Pan, Jeong Hyun Kim, Do-Young Yum
Background and Aims: Commercial superoxide dismutase (SOD) is derived from melon extract and has a potential as a dietary supplement due to its beneficial anti-oxidative effects. We aimed to improve the productivity of SOD compared with plant SOD by using a generally-regarded-as-safe (GRAS) microorganism, Bacillus amyloliquefaciens, and assess its anti-oxidative effect using γ-radiation- and dextransulfate sodium (DSS)-induced oxidative models in mice. Methods: We identified the sodA gene encoding manganese-containing SODs (Mn-SOD) in B...
March 14, 2018: Journal of Crohn's & Colitis
Grace S Chng, Dongdong Li, Chi Meng Chu, Tabitha Ong, Felicia Lim
Children who enter the child protection system often have complex family problems and have experienced early adverse experiences. Using latent class analysis, this study aimed to identify family classes of child protection cases in Singapore, to ascertain the prevalence of these family classes, and to test the association of family class membership to subsequent recurrence of harm. A sample of 440 cases who entered the Child Protective Service in Singapore was analyzed based on eight familial factors on the household and caregiver levels...
March 13, 2018: Child Abuse & Neglect
Judy F C Chow, William S B Yeung, Vivian C Y Lee, Estella Y L Lau, Ernest H Y Ng
OBJECTIVES: To evaluate the applicability of a commonly used next generation sequencing workflow in detecting unbalanced meiotic segregation products for reciprocal translocation and inversion carriers. STUDY DESIGN: All preimplantation genetic testing treatment cycles performed for reciprocal translocation or inversion carriers from 2012 to April 2017 were included. Three hundreds and forty-two archived whole genome amplified DNA, which had previously analyzed by array comparative genomic hybridization (aCGH), were retrospectively analyzed by next generation sequencing (NGS)...
March 9, 2018: European Journal of Obstetrics, Gynecology, and Reproductive Biology
Clio A Andreae, Richard B Sessions, Mumtaz Virji, Darryl J Hill
Neisseria meningitidis is an antigenically and genetically variable Gram-negative bacterium and a causative agent of meningococcal meningitis and septicaemia. Meningococci encode many outer membrane proteins, including Opa, Opc, Msf, fHbp and NadA, identified as being involved in colonisation of the host and evasion of the immune response. Although vaccines are available for the prevention of some types of meningococcal disease, none currently offer universal protection. We have used sequences within the Neisseria PubMLST database to determine the variability of msf and opc in 6,500 isolates...
2018: PloS One
Ya Chen, Yong Tang, Beibei Mao, Wenchao Li, Hongwei Jin, Liangren Zhang, Zhenming Liu
Any type of breast cancer not expressing genes of the estrogen receptor (ER), progesterone receptor (PR), or human epidermal growth factor receptor 2 (HER2) is referred to as triple-negative breast cancer (TNBC). Accordingly, TNBCs do not respond to hormonal therapies or medicines targeting the ER, PR, or HER2. Systemic chemotherapy is therefore the only treatment option available today and prognoses remain poor. We report the discovery and characterization of N-(naphtho[1,2-b]furan-5-yl)benzenesulfonamides as selective inhibitors of TNBCs...
March 16, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
Wishal D Ramdas, Jan S A G Schouten, Carroll A B Webers
BACKGROUND: The aim of is to determine the association of vitamins with glaucoma by performing a systematic review and meta-analyses. METHODS: Studies on the relation of vitamins and glaucoma published up to December 2017 were identified in the PubMed and Embase database. Data on vitamins (method of assessment), glaucoma (type and method of assessment), study characteristics and quality were recorded. In case of multiple studies for one nutrient a meta-analysis was performed...
March 16, 2018: Nutrients
Kazuma Takashima, Takashi Sakai, Hidetoshi Hamada, Masaki Takao, Nobuhiko Sugano
BACKGROUND: Many studies have confirmed that the size and location of necrotic lesions are major factors that affect the prevalence of collapse and prognosis in patients with osteonecrosis of the femoral head (ONFH). Although several classification systems categorize and quantify ONFH, there is no agreement on which one is most useful for the purpose. QUESTIONS/PURPOSES: We compared the Steinberg, modified Kerboul, and Japanese Investigation Committee (JIC) classifications of ONFH in terms of (1) the correlation among the three different classification systems...
March 15, 2018: Clinical Orthopaedics and related Research
William Martinez, James W Pichert, Gerald B Hickson, Casey H Braddy, Amy J Brown, Thomas F Catron, Ilene N Moore, Morgan R Stampfle, Lynn E Webb, William O Cooper
OBJECTIVES: The aims of the study were to develop a valid and reliable taxonomy of coworker reports of alleged unprofessional behavior by physicians and advanced practice professionals and determine the prevalence of reports describing particular types of unprofessional conduct. METHODS: We conducted qualitative content analysis of coworker reports of alleged unprofessional behavior by physicians and advanced practice professionals to create a standardized taxonomy...
March 15, 2018: Journal of Patient Safety
Sofia Doello, Zhibin Liang, Il Kyu Cho, Jung Bong Kim, Qing X Li
Lung cancer is the second most prevalent cancer. Non-small cell lung cancer (NSCLC) is the most common type of lung cancer. The low efficacy in current chemotherapies impels to find new alternatives to prevent or treat NSCLC. Rice bran oil is cytotoxic to A549 cells, a NSCLC cell line. Here, we identified 24-methylenecyloartanyl ferulate (24-mCAF) as the main component responsible for the cytotoxicity in A549 cells. An iTRAQ-based quantitative proteomics analysis revealed that 24-mCAF inhibits cell proliferation and activates cell death and apoptosis...
March 16, 2018: Journal of Agricultural and Food Chemistry
Elizabeth Hernandez, Raymond Rowan, Martina Randall
Primary bone lymphoma is a rare disease, accounting for less than 5% of all extranodal lymphoma. Although the femur is cited as being the most common site, primary bone lymphoma is rare, accounting for less than 1% of all non-Hodgkin's lymphoma. Herein we present a case of diffuse B-cell-type malignant lymphoma manifested as a large soft-tissue mass of the leg, via metastasis of primary non-Hodgkin's lymphoma of the femur, which went untreated. We highlight the advantages of various imaging modalities used throughout the process of diagnosis and treatment because accurate and early diagnosis are essential...
January 2018: Journal of the American Podiatric Medical Association
Martin A Bewley, Richard C Budd, Eilise Ryan, Joby Cole, Paul Collini, Jennifer Marshall, Umme Kolsum, Gussie Beech, Richard D Emes, Irina Tcherniaeva, Guy A M Berbers, Sarah R Walmsley, Gavin Donaldson, Jadwiga A Wedzicha, Iain Kilty, William Rumsey, Yolanda Sanchez, Christopher E Brightling, Louise E Donnelly, Peter J Barnes, Dave Singh, Moira K B Whyte, David H Dockrell
RATIONALE: Previous studies have identified defects in bacterial phagocytosis by alveolar macrophages (AM) in patients with chronic obstructive pulmonary disease (COPD) but the mechanisms and clinical consequences remain incompletely defined. OBJECTIVES: To examine the effect of COPD on AM phagocytic responses and identify the mechanisms, clinical consequences and potential for therapeutic manipulation of these defects. METHODS: We isolated alveolar macrophages (AM) and monocyte-derived macrophages (MDM) from a cohort of COPD patients and controls within the MRC COPD-MAP consortium and measured phagocytosis of bacteria in relation to opsonic conditions and clinical features...
March 16, 2018: American Journal of Respiratory and Critical Care Medicine
Patricia Lacourt, Julio Soto, Hana Rumié, Roger Gejman, Juan Carlos Pattillo, Cristián García, Hernán García
INTRODUCTION: Juvenile granulosa cell tumors (JGCT) are very rare, especially in infants under the age of one. The most frequent presentation is with signs of precocious puberty. OBJECTIVE: Present an in fant with peripheral precocious puberty, diagnosis of JGCT and follow up. CLINICAL CASE: 10-month-old female infant with thelarche, pubic hair and palpable abdominal mass accompanied with eleva ted levels of estradiol, very low gonadotrophins and images that show a very large ovarian mass...
December 2017: Revista Chilena de Pediatría
D Valenzuela, F Moya, M Luco, J L Tapia
50 years ago, Northway described Broncopulmonary Dysplasia (BPD) in preterm infants exposed to mechanical ventilation. Since then, their survival has increased, nevertheless a "new BPD" has appeared and its incidence has not diminished. One of the characteristics of this pathology is the the abnormal vascular remodeling, which in its most severe expression is known as Pulmonary Hyper tension (PH); with an incidence of 17% in patients with BPD, which is proportional to the severity of the disease (33% in severe BPD), and as mortality factor (up to 48% 2-year mortality in PH-BPD)...
December 2017: Revista Chilena de Pediatría
Jacob B Williams, Clara G Buchanan, William G Pitt
Background - Patients undergoing chemotherapy can develop resistance not only to the administered drug, but also to many other unrelated types of drugs, a phenomenon known as multidrug resistance. One of the most common mechanisms of multidrug resistance is an elevated expression of drug efflux pumps. Co-delivery of an efflux pump inhibitor with a chemotherapeutic can increase the killing of multidrug resistant cancer cells. Objective - Our hypothesis was that delivering doxorubicin directly to the cytosol of multidrug resistant cancer cells via a folate-targeted liposome loaded with a perfluoropentane emulsion droplet and doxorubicin (folated eLipoDox), along with the delivery of verapamil to block the efflux pumps will prove to be more effective at killing multidrug resistant cancer cells compared to conventional drug delivery...
March 16, 2018: Pharmaceutical Nanotechnology
Prashant Rao, Jack B Keenan, Taufiek K Rajab, Samuel Kim, Richard Smith, Orazio Amabile, Zain Khalpey
INTRODUCTION: Cardiovascular complications represent the leading cause of morbidity and mortality in patients with Marfan syndrome. Here, we describe a unique case where a total artificial heart was implanted in a young Marfan syndrome woman. METHODS: A 22-year-old postpartum African American female with Marfan syndrome developed multiple severe valve dysfunction and biventricular failure that was refractory to medical management. She previously had a Bentall procedure for Type A aortic dissection and repair of a Type B dissection...
March 2018: International Journal of Artificial Organs
Marco Guarene, Carla Badulli, Anna L Cremaschi, Ilaria Sbarsi, Rosalia Cacciatore, Carmine Tinelli, Annamaria Pasi, Paola Bergamaschi, Cesare G Perotti
INTRODUCTION: Allele-level donor-recipient match at HLA-A, HLA-B, HLA-C and HLA-DRB1 loci impacts the outcome after cord blood transplantation for hematologic malignancies and modifies the strategy of donor selection. High definition of both class I and II HLA loci at time of listing is a way to improve the attractiveness of cord blood bank inventories, reducing the time for donor search and procurement and simplifying donor choice, in particular, for patients of non-European heritage...
March 1, 2018: International Journal of Artificial Organs
Michael Mendler, Stefan Kopf, Jan B Groener, Christin Riedinger, Thomas H Fleming, Peter P Nawroth, Jürgen G Okun
AIMS: 5-Aminoimidazole-4-carboxamide riboside (AICAR) is an endogenous activator of AMPK, a central regulator of energy homeostasis. Loss and/or reduction of AMPK signaling plays an important role in the development of insulin resistance in type 2 diabetes. The loss of AMPK in diabetes could be due to a loss of AICAR. The aim of this study was to characterize urine levels of AICAR in diabetes and determine whether an association exists with respect to late complications, e.g., retinopathy, nephropathy and neuropathy...
March 15, 2018: Acta Diabetologica
Suhong Xiao, Ying-Sheng Tang, Praveen Kusumanchi, Sally P Stabler, Ying Zhang, Asok C Antony
Background: Epidemiologic and in vitro studies suggest independent linkages between poor folate and/or vitamin B-12 nutrition, genomic human papillomavirus (HPV) type 16 viral integration, and cancer. However, there is no direct evidence in vivo to support the causative role of poor folate nutrition in HPV16 integration into the cellular genome. Objective: We tested the hypothesis that folate deficiency enables the integration of HPV16 into the genome of HPV16-harboring keratinocytes, and could thereby influence earlier transformation of these cells to cancer in an animal model...
March 1, 2018: Journal of Nutrition
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