Results 91 to 100 of about 18,701 (299)

Early Clinical, Imaging, and Pathological Characteristics of SRPK3/TTN‐Digenic Myopathy

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective SRPK3/TTN‐digenic myopathy was recently established as a skeletal muscle myopathy caused by digenic inheritance. This study characterizes the early clinical presentation of SRPK3/TTN‐digenic myopathy in one previously reported and seven newly identified pediatric patients.
Rotem Orbach, Sandra Donkervoort, Carola Hedberg‐Oldfors, Giovanni Baranello, Dimah Saade, Precilla D'Souza, Ruchee Patel, Eva Michael, A. Reghan Foley, Diana Bharucha‐Goebel, S. Jin Haugland, Meghan McAnally, Omer Abdul Hamid, Katherine Chao, Ellen F. Macnamara, Alan H. Beggs, Anna Sarkozy, Juliane Mueller, Steven A. Moore, Richard S. Finkel, Cynthia J. Tifft, Francesco Muntoni, Anders Oldfors, Carsten G. Bönnemann   +23 more
wiley   +1 more source

A filamentation instability for streaming cosmic rays [PDF]

, 2011
We demonstrate that cosmic rays form filamentary structures in the precursors of supernova remnant shocks due to their self-generated magnetic fields.
Bell, A., B. Reville, A. R. Bell, Bell, A.R., Reville, B.   +4 more
core   +1 more source

Re‐Purposing Sapropterin (Kuvan) for ACTA2‐Related Multisystemic Smooth Muscle Dysfunction Syndrome: A Translational Mechanistic and First‐In‐Human Therapeutic Report

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Multisystemic smooth muscle dysfunction syndrome (MSMDS) is an ultra‐rare, ACTA2‐related disorder characterized by severe cerebrovascular disease, aortic aneurysms, and smooth muscle dysfunction. Using molecular dynamics simulations and in silico drug screening, we identified that sapropterin dihydrochloride (Kuvan) is a candidate capable of ...
Moran Hausman‐Kedem, Noa Bielopolski, Vijai Krishnan, Shane Wald‐Altman, Shelly I Shrian, Omer Bar‐Yosef, Livia Kapusta, Claire Shamber, Patricia L. Musolino, Miguel Weil   +9 more
wiley   +1 more source

Evaluation of Dried Plasma Spot‐Based Quantification of Glial Fibrillary Acidic Protein as a Disease‐Associated Biomarker in Neuromyelitis Optica Spectrum Disorder

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To evaluate the diagnostic accuracy of glial fibrillary acidic protein (GFAP) measured in dried plasma spots versus conventional plasma‐ and serum‐GFAP testing for assessment of disease severity in aquaporin‐4 immunoglobulin G–positive neuromyelitis optica spectrum disorder (AQP4‐IgG+ NMOSD).
Felix Wohlrab, Evelyn Alvarez, Roua Hamdi, Bingqing Zhang, Hayeun Ji, Niyati Jhaveri, Patrick Schindler, Pedro Sanchez, Pia S. Sperber, Tanja Schmitz‐Hübsch, Frederike C. Oertel, Nisa Vorasoot, Najib Kissani, Falko Böhringer, Jens Kuhle, Anne‐Katrin Pröbstel, Xiao‐Jun Ma, Péter Körtvelyessy, Patrick Waters, Friedemann Paul   +19 more
wiley   +1 more source

Ribosome deficiency induces Salmonella filamentation within host cells

mBio
The ribosome is the central hub for protein synthesis and is heavily targeted by antibiotics. Ribosomal mutations, antibiotic treatment, and nutrient starvation can alter translational efficiency and lead to stressed cells.
Zhihui Lyu, Cierra Wilson, Kalyn Weiss, Spencer Lewis, Kurt Fredrick, William Margolin, Jiqiang Ling   +6 more
doaj   +1 more source

Filamentation without intensity clamping

, 2010
We present measurements of the supercontinuum emission (SCE) from ultrashort Ti:Saph laser pulse filamentation in air in a tightly focused geometry. The spectral broadening of SCE indicates that peak intensities exceed the clamping value of a few 1013 W ...
Couairon, Arnaud, P. Prem Kiran, Kumar, G. Ravindra, Suman Bagchi, Arnaud Couairon, Arnold, Cord L., Bagchi, Suman, Cord L. Arnold, Siva Rama Krishnan, G. Ravindra Kumar, Prem Kiran, P., Krishnan, Siva Rama   +11 more
core   +1 more source

Mechanochemical Synthesis and Characterization of Nanostructured ErB4 and NdB4 Rare‐Earth Tetraborides

Advanced Engineering Materials, Volume 27, Issue 6, March 2025.
ErB4 and NdB4 nanostructured powders are produced by mechanochemical synthesis. 5 h mechanical alloying and 4 M HCl acid leaching are used in the production. ErB4 and NdB4 powders exhibit maximum magnetization of 0.4726 emu g−1 accompanied with an antiferromagnetic‐to‐paramagnetic phase transition at about TN = 18 K and 0.132 emu g−1 with a maximum at ...
Burçak Boztemur, Faruk Kaya, Bora Derin, Mustafa Lütfi Öveçoğlu, Ju Li, Duygu Ağaoğulları   +5 more
wiley   +1 more source

Comparison of Triply Periodic Minimal Surface Energy Absorbers Under Uniaxial Compressive Loading

Advanced Engineering Materials, EarlyView.
This study investigates LCD 3D printed Triply Periodic Minimal Surface (TPMS) structures as mechanical energy absorbers. By comparing various base designs and layered combinations under uniaxial compression, it identifies that a Diamond‐Gyroid sandwich structure offers superior performance.
Sergej Grednev, Noah‐Lean Roschlaub, Anne Jung   +2 more
wiley   +1 more source

Enhancement of the Through‐Thickness Electrical Conductivity of Carbon‐Fiber‐Reinforced Plastics Using Large Graphite Particles

Advanced Engineering Materials, EarlyView.
To enhance through‐thickness conductivity without sacrificing impregnation, large spherical graphite particles are intentionally employed in a low‐viscosity resin. Unlike finer conductive fillers, these particles remain outside the fiber bundles and accumulate in resin‐rich interlaminar regions during molding.
Keito Hosoe, Yu Zhou, Shintaro Kamiyama, Takao Okada, Tomohiro Yokozeki, Ryoma Tokonami, Tatsuhiro Takahashi   +6 more
wiley   +1 more source

Roles of P-body factors in Candida albicans filamentation and stress response.

PLoS Genetics
Hyphal growth is strongly associated with virulence in the human fungal pathogen Candida albicans. While hyphal transcriptional networks have been the subject of intense study, relatively little is known about post-transcriptional regulation.
Melissa A Tosiano, Frederick Lanni, Aaron P Mitchell, C Joel McManus   +3 more
doaj   +1 more source