Results 71 to 80 of about 195,193 (266)

A minimal cellulosome‐like system in Cellulosilyticum lentocellum

FEBS Open Bio, EarlyView.
Cellulose‐degrading bacteria typically use cellulosomes, large multi‐enzyme complexes on a scaffold protein. In Cellulosilyticum lentocellum, we characterise a far smaller arrangement, a single scaffold bound to one cellulase through a single cohesin‐dockerin interaction.
John Allan, Amias Alstrom‐Moore, Gary W Black   +2 more
wiley   +1 more source

Bile acids and bile alcohols in a child with hepatic 3 beta-hydroxy-delta 5-C27-steroid dehydrogenase deficiency: effects of chenodeoxycholic acid treatment

Journal of Lipid Research, 1991
Duodenal bile, urine, plasma, and feces from a child with hepatic 3 beta-hydroxy-delta 5-C27-steroid dehydrogenase deficiency were analyzed by fast atom bombardment mass spectrometry and gas chromatography-mass spectrometry to investigate the formation ...
H Ichimiya, B Egestad, H Nazer, ES Baginski, PT Clayton, J Sjövall   +5 more
doaj   +1 more source

Developmental and Epileptic Encephalopathy due to Biallelic Pathogenic Variants in PIGM

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective PIGM encodes a critical enzyme in the glycosylphosphatidylinositol (GPI)‐anchor biosynthesis pathway. While promoter‐region mutations in PIGM have been associated with a relatively mild phenotype characterized by portal vein thrombosis and absence seizures, recent evidence suggests that coding‐region mutations result in a more severe
Júlia Sala‐Coromina, Anna Marcé‐Grau, Barbara Masotto, Marta Codina, Lamia BenJemaa, Yasmina Elaribi, Mónica Martinez‐Gallo, Roger Colobran, Angel Sanchez‐Montañez, Irene Valenzuela, Yoshiko Murakami, Alfons Macaya   +11 more
wiley   +1 more source

Quantification of trace 227Ac and other radionuclidic impurities in mass-separated 225Ac samples produced at CERN-MEDICIS

Scientific Reports
225Ac is a promising candidate medical radionuclide for targeted alpha therapy of advanced stage cancers. One of the main production pathways is the high-energy proton spallation of thorium-based targets, that requires an efficient, nuclide-selective ...
Jake D. Johnson, Cyril Bernerd, Frank Bruchertseifer, Thomas E. Cocolios, Marie Deseyn, Charlotte Duchemin, Michael Heines, Max Keppens, Laura Lambert, Nathan Meurrens, Ralf E. Rossel, Thierry Stora, Viktor Van den Bergh   +12 more
doaj   +1 more source

Renal Amyloidosis Associated With 5 Novel Variants in the Fibrinogen A Alpha Chain Protein

Kidney International Reports, 2017
Fibrinogen A alpha chain amyloidosis is an autosomal dominant disease associated with mutations in the fibrinogen A alpha chain (FGA) gene, and it is the most common cause of hereditary renal amyloidosis in the UK.
Dorota Rowczenio, Maria Stensland, Gustavo A. de Souza, Erik H. Strøm, Janet A. Gilbertson, Graham Taylor, Nigel Rendell, Shane Minogue, Yvonne A. Efebera, Helen J. Lachmann, Ashutosh D. Wechalekar, Philip N. Hawkins, Ketil R. Heimdal, Kristian Selvig, Inger K. Lægreid, Nathalie Demoulin, Selda Aydin, Julian D. Gillmore, Tale N. Wien   +18 more
doaj   +1 more source

Plasma EV Proteomics Identifies ECM Remodeling and Inflammatory Proteins LUM and C7 as Candidate Biomarkers in FSHD

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Facioscapulohumeral muscular dystrophy (FSHD) is one of the most debilitating and common muscular dystrophies. Despite its severity, no approved therapy exists for FSHD patients. However, several therapeutic candidates are currently under development, and some have recently entered clinical trials, marking the need for reliable ...
Mustafa Bilal Bayazit, Chiranth K. Nagaraj, Jackson S. Newell, Kim Truc Nguyen, Xilal Y. Rima, Jacob Doon‐Ralls, Eduardo Reátegui, Jeffrey M. Statland, Rabi Tawil, Kevin M. Flanigan, Scott Q. Harper, Nizar Y. Saad   +11 more
wiley   +1 more source

Neurochemical Endpoints to Inform Early‐Stage Trials of Spinocerebellar Ataxia 2 and 3 in a Multisite Setting

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Neurochemical levels measured by brain MR spectroscopy (MRS) have been proposed as endpoints for clinical trials in early‐stage spinocerebellar ataxia (SCA) trials. We tested their trial‐readiness by quantifying neurochemicals in three affected brain regions in early‐stage cohorts of SCA2 and SCA3, examining their reproducibility in ...
James M. Joers, Yaqiao Wei, Dinesh K. Deelchand, Adam Berrington, Young Woo Park, Guita Banan, Michal Povazan, Jens T. Rosenberg, André J. W. van der Kouwe, Tetsuo Ashizawa, Khalaf O. Bushara, Chiadi U. Onyike, Sub H. Subramony, Jeremy D. Schmahmann, Brian J. Soher, Peter B. Barker, Thomas H. Mareci, Eva‐Maria Ratai, Lynn E. Eberly, Gülin Öz   +19 more
wiley   +1 more source

Absolute quantification of tumor necrosis factor-alpha by isotope dilution mass spectrometry

Frontiers in Chemistry
Tumor necrosis factor-α (TNF-α) is an important inflammatory mediator and has been widely recognized as a diagnostic biomarker for various autoimmune and infectious diseases in clinical practice, such as rheumatoid arthritis.
Chenchen Bao, Jing Wang, Wenqiang Xia, Ke Yang, Xinchang Shi, Xiaoying Yang, Hailin Li, Yuemin Wang, Yafen Zhang   +8 more
doaj   +1 more source

Rheumatoid Arthritis and Coronary Artery Calcium Progression: A Case Cohort Analysis From ELSA‐Brasil

Arthritis Care &Research, EarlyView.
Objective To investigate the association between rheumatoid arthritis (RA) and coronary artery calcium (CAC) prevalence, incidence, and progression over four years in adults without prior cardiovascular disease. Methods A case‐cohort study within the Brazilian Longitudinal Study of Adult Health (ELSA‐Brasil) included 585 participants (86 patients with ...
Patrícia Fonseca Estrada, Marcos Rafael N. Cavalcante, Itamar de Souza Santos, Márcio S. Bittencourt, Vandrize Meneghini, Paulo A. Lotufo, Alessandra C. Goulart, Isabela M. Bensenor   +7 more
wiley   +1 more source

Sequential separation of uranium and plutonium in urine sample using UTEVA resin

Radiation Protection and Environment
This article describes the process of separating uranium (U) and plutonium (Pu) sequentially in urine samples through the use of UTEVA resin. The manuscript optimizes several parameters, including the concentration of nitric acid in the loading solution,
Rupali Dubla, Ranjeet Kumar, Nanda Raveendran, J. R. Yadav, Pramilla D. Sawant   +4 more
doaj   +1 more source