Results 41 to 50 of about 58,003 (251)

Targeted Exon Skipping to Address “Leaky” Mutations in the Dystrophin Gene

Molecular Therapy: Nucleic Acids, 2012
Protein-truncating mutations in the dystrophin gene lead to the progressive muscle wasting disorder Duchenne muscular dystrophy, whereas in-frame deletions typically manifest as the milder allelic condition, Becker muscular dystrophy.
Sue Fletcher, Carl F. Adkin, Penny Meloni, Brenda Wong, Francesco Muntoni, Ryszard Kole, Clayton Fragall, Kane Greer, Russell Johnsen, Steve D. Wilton   +9 more
doaj   +1 more source

Dirty Fish Versus Squeaky Clean Mice: Dissecting Interspecies Differences Between Animal Models of Interferonopathy

Frontiers in Immunology, 2021
Autoimmune and autoinflammatory diseases are rare but often devastating disorders, underpinned by abnormal immune function. While some autoimmune disorders are thought to be triggered by a burden of infection throughout life, others are thought to be ...
Holly A. Rutherford, Paul R. Kasher, Paul R. Kasher, Noémie Hamilton   +3 more
doaj   +1 more source

A Unifying Thermodynamic Model for Phase Separation and Aging of Biopolymers

Advanced Science, EarlyView.
Phase separation and aging of intrinsically disordered proteins are placed in a unifying framework. A thermodynamically consistent time‐dependent version of associating‐polymer theory shows how the processes are intricately coupled. Assuming aging to occur through interacting sites resulting from reversible conformational transitions, the model ...
Jasper J. Michels, Joana Caria, Edward A. Lemke   +2 more
wiley   +1 more source

Canonical Antibodies Adopt Distinct Binding Modes to Recognize Viral Glycan Shields

Advanced Science, EarlyView.
Canonical Y‐shaped antibodies recognize viral glycan shields through adaptive Fab assembly states shaped by glycan organization and somatic hypermutation. Structural analyses ofbroadly neutralizing antibodies VRC35 and VRC36 across glycoproteins of HIV‐1, influenza, SARS‐CoV‐2, and Lassa viruses reveal distinct Fab assembly states, spanning monovalent,
Jiaxuan Cheng, Evan M. Cale, Amirabbas Maghsoudi, Matthew S. Sutton, Nancy S. Longo, Rebecca A. Gillespie, Lingshu Wang, Ivan Kosik, Sue Chong, Atsuhiro Yasuhara, Tatsiana Bylund, Arne Schön, Prabhanshu Tripathi, Yaroslav Tsybovsky, Haotian Lei, Nicholas C. Morano, Allison B. Lupatkin, Abraham J. Morton, Zabrina C. Lang, Jordan E. Becker, Isabella R. Frascilla, Myungjin Lee, Ning Li, Cuiping Liu, Ryan S. Roark, Chen‐Hsiang Shen, I‐Ting Teng, David J. Van Wazer, Danyi Wang, Lingyuan Wu, Goran Ahlsen, Mike Castro, Haijuan Du, Michael J. Ernandes, Tiansheng Li, Bob C. Lin, Mark K. Louder, Krisha McKee, Jonah S. Merriam, Sijy O'Dell, Li Ou, Sergei Pletnev, David Prikryl, Qi Qiu, Sarah Rubin, Mallika Sastry, Stephen D. Schmidt, Asif Shajahan, Andrea R. Shiakolas, Sanjay Srivatsan, Baoshan Zhang, Qiong Zhou, Mark Connors, Jason G. Gall, Yicheng Guo, Rick K. Huang, Yaoxing Huang, Richard A. Koup, Q. Paula Lei, John R. Mascola, Reda Rawi, Leonid Serebryannyy, Lawrence Shapiro, Zizhang Sheng, David D. Ho, Patrick C. Wilson, Jonathan W. Yewdell, Theodore C. Pierson, Masaru Kanekiyo, Nicole A. Doria‐Rose, Peter D. Kwong, Tongqing Zhou   +71 more
wiley   +1 more source

Vamorolone improves Becker muscular dystrophy and increases dystrophin protein in bmx model mice

iScience, 2023
Summary: There is no approved therapy for Becker muscular dystrophy (BMD), a genetic muscle disease caused by in-frame dystrophin deletions. We previously developed the dissociative corticosteroid vamorolone for treatment of the allelic, dystrophin-null ...
Nikki M. McCormack, Nhu Y. Nguyen, Christopher B. Tully, Trinitee Oliver, Alyson A. Fiorillo, Christopher R. Heier   +5 more
doaj   +1 more source

Promoting Healthier Drinking: Evidence From a Vignette Experiment on Contextual and Informational Drivers of Dealcoholized Wine Choices

Applied Economic Perspectives and Policy, EarlyView.
ABSTRACT Growing demand for healthier beverages is driving innovation in the wine sector, with dealcoholized wine emerging as a promising alternative. However, little is known about the contextual conditions under which consumers would choose dealcoholized wine, particularly in countries with strong wine traditions. To fill this gap, this work examines
Giovanna Piracci, Giulia Maesano, Leonardo Casini, Maurizio Canavari, Marija Cerjak   +4 more
wiley   +1 more source

Breast Reconstruction in Becker Nevus Syndrome

Plastic and Reconstructive Surgery, Global Open
Summary:. Becker nevus syndrome is a rare cutaneous disorder characterized by the presence of a hyperpigmented patch of skin typically occurring on the upper trunk, often associated with hypertrichosis, and sometimes associated with musculoskeletal ...
Alexis K. Schlosser, MD, Mohammed S. Rais, BS, Ruston J. Sanchez, MD   +2 more
doaj   +1 more source

Exonization of a deep intronic long interspersed nuclear element in Becker muscular dystrophy

Frontiers in Genetics, 2022
The precise identification of pathogenic DMD variants is sometimes rather difficult, mainly due to complex structural variants (SVs) and deep intronic splice-altering variants.
Zhiying Xie, Chang Liu, Yanyu Lu, Chengyue Sun, Yilin Liu, Meng Yu, Junlong Shu, Lingchao Meng, Jianwen Deng, Wei Zhang, Zhaoxia Wang, He Lv, Yun Yuan   +12 more
doaj   +1 more source

Assessing Haitian Consumers' Willingness to Pay a Premium for Aflatoxin‐Compliant Peanut Butter in the Informal Market

Agribusiness, EarlyView.
ABSTRACT Market‐based solutions are increasingly tested to address aflatoxin issues in peanuts in developing countries. Although previous studies have found that Haitian grocery store shoppers are willing to pay a 21% premium for peanut butter with levels of aflatoxin that meet international standards, no information is available for the much larger ...
Phendy Jacques, Stevens Azima, Maurice Doyon   +2 more
wiley   +1 more source

How does the Kids SIPsmartER program impact the sugar‐sweetened beverage intake of students: An investigation beyond total treatment effect in randomized controlled trial

American Journal of Agricultural Economics, EarlyView.
Abstract This study develops and empirically estimates a structural framework to decompose the causal pathways of multilevel behavioral interventions targeting adolescent health behaviors. We apply this framework to the Kids SIPsmartER (KSS) program, a 6‐month, school‐based intervention evaluated through a clustered randomized controlled trial in rural
Naveen Abedin, Wen You, Annie Reid, Kathleen Porter, Brittany Kirkpatrick, Jamie Zoellner   +5 more
wiley   +1 more source