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https://www.readbyqxmd.com/read/28213126/the-galactose-induced-decrease-in-phosphate-levels-leads-to-toxicity-in-yeast-models-of-galactosemia
#1
Caio M Machado, Evandro A De-Souza, Ana Luiza F V De-Queiroz, Felipe S A Pimentel, Guilherme F S Silva, Fabio M Gomes, Mónica Montero-Lomelí, Claudio A Masuda
Classic galactosemia is an inborn error of metabolism caused by deleterious mutations in the GALT gene. A number of evidences indicate that the galactose-1-phosphate accumulation observed in patient cells is a cause of toxicity in this disease. Nevertheless, the consequent molecular events caused by the galactose-1-phosphate accumulation remain elusive. Here we show that intracellular inorganic phosphate levels decreased when yeast models of classic galactosemia were exposed to galactose. The decrease in phosphate levels is probably due to the trapping of phosphate in the accumulated galactose-1-phosphate since the deletion of the galactokinase encoding gene GAL1 suppressed this phenotype...
February 14, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28173647/-analysis-of-newborn-screening-for-galactosemia-and-genotype-phenotype-of-confirmed-galatosemia-cases
#2
R L Yang, F Tong, F Hong, G L Qian, D W Wu, Z Y Zhao
Objective: To investigate the prevalence of galactosemia(GAL), and the characteristics of genotype and phenotype of newborns who were confirmed with GAL in newborn screening in Zhejiang province. Method: The number of all live births, newborn screened infants and all clinical data of confirmed newborns with GAL from October 2013 to March 2015 were retrospectively analyzed by reviewing the data of Zhejiang Province screening center database. And the characteristics of genes and the clinical data of GAL cases who were confirmed by correlative gene test and enzyme activity measurement were analyzed...
February 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28166712/experimental-acute-infection-of-alpacas-with-bovine-viral-diarrhea-virus-1-subgenotype-b-alters-peripheral-blood-and-galt-leukocyte-subsets
#3
Christina L Topliff, Abdulrahman A Alkheraif, Charles A Kuszynski, William C Davis, David J Steffen, Jack A Schmitz, Kent M Eskridge, Bryan Charleston, Jamie N Henningson, Clayton L Kelling
Bovine viral diarrhea virus (BVDV) is a pathogen in cattle and alpacas ( Vicugna pacos), causing acute and persistent BVDV infections. We characterized the effect of acute BVDV infection on the immune system of alpacas by determining lymphocyte subpopulations in peripheral blood and gut-associated lymphoid tissues (GALT) as well as serum interferon levels. Alpacas were experimentally infected with BVDV-1b (strain CO-06). Peripheral blood leukocytes were isolated at 0, 3, 6, and 9 d postinfection (dpi), and leukocytes of GALT at 9 dpi, and evaluated using flow cytometry...
January 1, 2017: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/28154012/gastrointestinal-immune-and-microbiome-changes-during-parenteral-nutrition
#4
Joseph F Pierre
Parenteral Nutrition (PN) is a lifesaving therapy that provides intravenous nutrition support to patients who cannot, or should not, feed via the gastrointestinal tract. Unfortunately, PN also carries certain risks related to infection and metabolic complications compared with enteral nutrition. In this review, an overview of PN and gastrointestinal (GI) immune and microbiome changes is provided. PN impacts the gut-associated lymphoid tissue (GALT) functions - especially adaptive immune cells - changes to intestinal epithelium and chemical secretions, and significantly alters the intestinal microbiome...
February 2, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28117931/role-of-intrinsic-graft-vs-extrinsic-host-factors-in-the-growth-of-transplanted-organs-following-allogeneic-and-xenogeneic-transplantation
#5
Tatsu Tanabe, Hironosuke Watanabe, Jigesh A Shah, Hisashi Sahara, Akira Shimizu, Shunichiro Nomura, Arsenoi Asfour, Makenzie Danton, Lennan Boyd, Adrienne Dardenne Meyers, Dilrukshi K Ekanayake-Alper, David H Sachs, Kazuhiko Yamada
In our studies of life-supporting GalT-KO pig-to-baboon kidneys, we have found that some recipients developed increased serum creatinine with growth of the grafts, without histologic or immunologic evidence of rejection. We hypothesized that the rapid growth of orthotopic pig grafts in smaller baboon recipients may have led to deterioration of organ function. To test this hypothesis for both kidneys and lungs, we have assessed whether the growth of outbred (Yorkshire) organ transplants in miniature swine is regulated by intrinsic (graft) or extrinsic (host environment) factors...
January 24, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28095018/hallway-conversations-in-physicswhat-is-the-typical-effective-dose-from-a-routine-eye-to-thigh-pet-ct-examination-assuming-an-injected-18-f-fdg-dose-of-370-mbq
#6
Shalmali Dharmadhikari, James R Galt, Jonathon A Nye
No abstract text is available yet for this article.
January 17, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28087077/alteration-of-cd39-foxp3-cd4-t-cell-and-cytokine-levels-in-eae-ms-following-anti-cd52-treatment
#7
Anudeep B Pant, Yan Wang, Daniel W Mielcarz, Eli J Kasper, Kiel M Telesford, Megan Mishra, Azizul Haque, Jacqueline Smith, Lloyd H Kasper, Sakhina Begum-Haque
While examining the therapeutic value of anti-CD52 antibody against EAE/MS, we identified a unique subset of CD39+ Tregs in repopulating GALT tissues, a major lymphoid reservoir, which was accompanied by amelioration of disease. Furthermore, anti-CD52 treatment leads to increased expression of BDNF, IL-10, and SMAD3 in the brains of EAE mice. This condition is associated with suppression of IL-17, a critical inflammatory factor in EAE/MS progression. Additionally, we found elevated levels of CD4+CD39+ Tregs in PBMCs of RRMS patients treated with humanized anti-CD52 mAb...
December 21, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28078493/false-positive-newborn-screen-using-the-beutler-spot-assay-for-galactosemia-in-glucose-6-phosphate-dehydrogenase-deficiency
#8
Grace Stuhrman, Stefanie J Perez Juanazo, Kea Crivelly, Jennifer Smith, Hans Andersson, Eva Morava
Classical galactosemia is detected through newborn screening by measuring galactose-1-phosphate uridylyltransferase (GALT) in the USA primarily via the Beutler spot assay. We report on an 18-month-old patient with glucose-6-phosphate dehydrogenase (G6PD) deficiency that was originally diagnosed with classical galactosemia. The patient presented with elevated liver function enzymes and bilirubinemia and was immediately treated with soy-based formula. Confirmatory tests revealed deficiency of the GALT enzyme, however, full-sequencing of GALT was normal, suggestive of a different ideology...
January 12, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28075131/classical-galactosemia-insight-into-molecular-pathomechanisms-by-differential-membrane-proteomics-of-fibroblasts-under-galactose-stress
#9
Simon Staubach, Stefan Müller, Murat Pekmez, Franz-Georg Hanisch
Classical galactosemia, a hereditary metabolic disease caused by the deficiency of galactose-1-phosphate uridyltransferase (GALT; EC 2.7.712), results in an impaired galactose metabolism and serious long-term developmental affection of the CNS and ovaries, potentially related in part to endogenous galactose-induced protein dysglycosylation. In search for galactose-induced changes in membrane raft proteomes of GALT-deficient cells, we performed differential analyses of lipid rafts from patient-derived (Q) and sex- and age-matched control fibroblasts (H) in the presence or absence of the stressor...
January 24, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/28065439/nine-years-of-newborn-screening-for-classical-galactosemia-in-the-netherlands-effectiveness-of-screening-methods-and-identification-of-patients-with-previously-unreported-phenotypes
#10
Lindsey Welling, Anita Boelen, Terry G J Derks, Peter C J I Schielen, Maaike de Vries, Monique Williams, Frits A Wijburg, Annet M Bosch
INTRODUCTION: Newborn screening (NBS) for classical galactosemia (CG) was introduced in the Netherlands in 2007. Multiple screening methods have been used since, and currently a two-tier system is used, with residual enzyme activity of galactose-1-phosphate-uridyltransferase (GALT) and total galactose concentration in dried blood spots as the primary and secondary markers. As it is essential to monitor effectiveness of NBS programs, we assessed the effectiveness of different screening methods used over time (primary aim), and aimed to identify and investigate patients identified through NBS with previously unreported clinical and biochemical phenotypes (secondary aim)...
December 29, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28018327/correlation-of-gut-microbiota-composition-with-resistance-to-experimental-autoimmune-encephalomyelitis-in-rats
#11
Suzana Stanisavljević, Jovanka Lukić, Svetlana Soković, Sanja Mihajlovic, Marija Mostarica Stojković, Djordje Miljković, Natasa Golić
Multiple sclerosis is a chronic inflammatory disease of the central nervous system (CNS). It is widely accepted that autoimmune response against the antigens of the CNS is the essential pathogenic force in the disease. It has recently become increasingly appreciated that activated encephalitogenic cells tend to migrate toward gut associated lymphoid tissues (GALTs) and that interrupted balance between regulatory and inflammatory immunity within the GALT might have decisive role in the initiation and propagation of the CNS autoimmunity...
2016: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28011469/three-pathways-of-colonic-carcinogenesis-in-rats
#12
REVIEW
Carlos A Rubio
Colorectal cancer (CRC) is one of the more intensively studied human malignancies. For many years, the general view has been that the vast majority of CRCs in humans evolve from conventional (tubular or villous) adenomas via the adenoma-carcinoma pathway. More recently, serrated colorectal polyps (hyperplastic polyps, sessile serrated polyps and traditional serrated adenomas) have emerged as an alternative pathway of colorectal carcinogenesis in humans. Archival sections from early experiments in Sprague-Dawley (SD) rats injected with dimethylhydrazine (DMH) were reviewed and the histology of colonic neoplasias was re-evaluated...
2017: Anticancer Research
https://www.readbyqxmd.com/read/27960097/fluorescent-mannosides-serve-as-acceptor-substrates-for-glycosyltransferase-and-sugar-1-phosphate-transferase-activities-in-euglena-gracilis-membranes
#13
Irina M Ivanova, Sergey A Nepogodiev, Gerhard Saalbach, Ellis C O'Neill, Michael D Urbaniak, Michael A J Ferguson, Sudagar S Gurcha, Gurdyal S Besra, Robert A Field
Synthetic hexynyl α-D-mannopyranoside and its α-1,6-linked disaccharide counterpart were fluorescently labelled through CuAAC click chemistry with 3-azido-7-hydroxycoumarin. The resulting triazolyl-coumarin adducts, which were amenable to analysis by TLC, HPLC and mass spectrometry, proved to be acceptor substrates for α-1,6-ManT activities in mycobacterial membranes, as well as α- and β-GalT activities in trypanosomal membranes, benchmarking the potential of the fluorescent acceptor approach against earlier radiochemical assays...
January 13, 2017: Carbohydrate Research
https://www.readbyqxmd.com/read/27942870/new-insights-in-the-analysis-of-blunt-force-trauma-in-human-bones-preliminary-results
#14
Sarah Scheirs, Assumpció Malgosa, David Sanchez-Molina, Marisa Ortega-Sánchez, Joan Velázquez-Ameijide, Carlos Arregui-Dalmases, Jordi Medallo-Muñiz, Ignasi Galtés
Determining the time of injury is an important but still a challenging task in forensic anthropology. In literature, many descriptions can be found to make a distinction between perimortem and postmortem fractures. Characteristics that are more related to fractures in fresh conditions, however, are not extensively investigated. This study compared 28 perimortem fractures from autopsies and 21 both fresh and dry experimentally reproduced human bone fractures. Preliminary results showed the following five distinct traits that might be related to perimortem conditions: layered breakage, bone scales, crushed margins, wave lines and flakes with matching flake defect...
December 10, 2016: International Journal of Legal Medicine
https://www.readbyqxmd.com/read/27939424/enhanced-mucosal-defense-and-reduced-tumor-burden-in-mice-with-the-compromised-negative-regulator-irak-m
#15
Daniel E Rothschild, Yao Zhang, Na Diao, Christina K Lee, Keqiang Chen, Clayton C Caswell, Daniel J Slade, Richard F Helm, Tanya LeRoith, Liwu Li, Irving C Allen
Aberrant inflammation is a hallmark of inflammatory bowel disease (IBD) and colorectal cancer. IRAK-M is a critical negative regulator of TLR signaling and overzealous inflammation. Here we utilize data from human studies and Irak-m(-/-) mice to elucidate the role of IRAK-M in the modulation of gastrointestinal immune system homeostasis. In human patients, IRAK-M expression is up-regulated during IBD and colorectal cancer. Further functional studies in mice revealed that Irak-m(-/-) animals are protected against colitis and colitis associated tumorigenesis...
February 2017: EBioMedicine
https://www.readbyqxmd.com/read/27919945/the-leloir-pathway-of-galactose-metabolism-a-novel-therapeutic-target-for-hepatocellular-carcinoma
#16
Manshu Tang, Enoabasi Etokidem, Kent Lai
Hepatocellular carcinoma (HCC) is one of the most lethal types of cancer worldwide, with poor prognosis and limited treatments. In order to identify novel therapeutic targets that will lead to development of effective therapies with manageable side effects, we tested the hypothesis that knocking-down galactokinase (GALK1) or galactose-1 phosphate uridylyltransferase (GALT) gene expression would control the growth of cultured hepatoma cells. Our results showed small interfering RNA (siRNA) against GALK1 or GALT inhibited the growth of HepG2 cells in culture...
2016: Anticancer Research
https://www.readbyqxmd.com/read/27899642/the-ucsc-genome-browser-database-2017-update
#17
Cath Tyner, Galt P Barber, Jonathan Casper, Hiram Clawson, Mark Diekhans, Christopher Eisenhart, Clayton M Fischer, David Gibson, Jairo Navarro Gonzalez, Luvina Guruvadoo, Maximilian Haeussler, Steve Heitner, Angie S Hinrichs, Donna Karolchik, Brian T Lee, Christopher M Lee, Parisa Nejad, Brian J Raney, Kate R Rosenbloom, Matthew L Speir, Chris Villarreal, John Vivian, Ann S Zweig, David Haussler, Robert M Kuhn, W James Kent
Since its 2001 debut, the University of California, Santa Cruz (UCSC) Genome Browser (http://genome.ucsc.edu/) team has provided continuous support to the international genomics and biomedical communities through a web-based, open source platform designed for the fast, scalable display of sequence alignments and annotations landscaped against a vast collection of quality reference genome assemblies. The browser's publicly accessible databases are the backbone of a rich, integrated bioinformatics tool suite that includes a graphical interface for data queries and downloads, alignment programs, command-line utilities and more...
January 4, 2017: Nucleic Acids Research
https://www.readbyqxmd.com/read/27898686/gut-homing-cd4-and-cd8-t-cell-frequencies-in-hiv-infected-individuals-on-antiretroviral-treatment
#18
Olivia Briceño, Sandra Pinto-Cardoso, Nataly Rodríguez-Bernabe, Akio Murakami-Ogasawara, Gustavo Reyes-Terán
The depletion of mucosal CD4+ T-cells occurs early in HIV infection and despite years on antiretroviral treatment (ART), this population never reconstitutes to pre-HIV infection levels. In an effort to understand the effect of ART initiation and different ART regimens on the reconstitution of mucosal T cells within the gut associated lymphoid tissue (GALT), we quantified the frequency of CD4+ and CD8+ T cells expressing the gut homing receptors CCR9 and β7 in peripheral blood (PB) of HIV infected individuals naive to ART and treated individuals on both short-term (less than a year) and long-term ART (more than 2 years)...
2016: PloS One
https://www.readbyqxmd.com/read/27888523/the-cotton-%C3%AE-galactosyltransferase-1-galt1-that-galactosylates-arabinogalactan-proteins-participates-in-controlling-fiber-development
#19
Li-Xia Qin, Yun Chen, Wei Zeng, Yang Li, Lu Gao, Deng-Di Li, Antony Bacic, Wen-Liang Xu, Xue-Bao Li
Arabinogalactan-proteins (AGPs) are highly glycosylated proteins which play pivotal roles in diverse developmental processes in plants. Type II AG glycans, mostly O-linked to the hydroxyproline residues of the protein backbone, account for up to 95% w/w of the AGP, but their functions are still largely unclear. Cotton fibers are extremely elongated single-cell trichomes on the seed epidermis. However, little is known of the molecular basis governing the regulation of fiber cell development. Here, we characterized the role of a CAZy glycosyltransferase (GT) family 31 member GhGalT1 in cotton fiber development...
November 26, 2016: Plant Journal: for Cell and Molecular Biology
https://www.readbyqxmd.com/read/27869292/model-based-investigation-of-intracellular-processes-determining-antibody-fc-glycosylation-under-mild-hypothermia
#20
Si Nga Sou, Philip M Jedrzejewski, Ken Lee, Christopher Sellick, Karen M Polizzi, Cleo Kontoravdi
Despite the positive effects of mild hypothermic conditions on monoclonal antibody (mAb) productivity (qmAb ) during mammalian cell culture, the impact of reduced culture temperature on mAb Fc-glycosylation and the mechanism behind changes in the glycan composition is not fully established. The lack of knowledge about the regulation of dynamic intracellular processes under mild hypothermia restricts bioprocess optimisation. To address this issue, a mathematical model that quantitatively describes CHO cell behaviour and metabolism, mAb synthesis and its N-linked glycosylation profiles before and after the induction of mild hypothermia is constructed using two sets of parameters...
November 21, 2016: Biotechnology and Bioengineering
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