A5083328627- Muscle Physiology and Disorders
- Virus-based gene therapy research
- Adipose Tissue and Metabolism
- Ion channel regulation and function
- Genetic Neurodegenerative Diseases
- Ubiquitin and proteasome pathways
- Cellular transport and secretion
- Cardiomyopathy and Myosin Studies
- Neuroscience and Neuropharmacology Research
- Neurogenetic and Muscular Disorders Research
- Liver Disease Diagnosis and Treatment
- Neurobiology and Insect Physiology Research
- Muscle metabolism and nutrition
- Silk-based biomaterials and applications
- Neurotransmitter Receptor Influence on Behavior
- Cell Adhesion Molecules Research
- Tissue Engineering and Regenerative Medicine
- Lipid Membrane Structure and Behavior
- Nerve injury and regeneration
- Exercise and Physiological Responses
- Drug Transport and Resistance Mechanisms
- Viral Infectious Diseases and Gene Expression in Insects
- Viral Infections and Immunology Research
- CRISPR and Genetic Engineering
- Biotin and Related Studies
Nationwide Children's Hospital
2014-2024
The Ohio State University
2014-2024
Cornell University
2019
Boise State University
2017-2018
Université d'Angers
2017
Centre Hospitalier Universitaire d'Angers
2017
Université Nantes Angers Le Mans
2017
Takeda (United States)
2009-2017
Royal Brisbane and Women's Hospital
2012-2015
University of Miami
2010-2015
The effects of product and preparation variables on the in-cup chemical composition tea extracts is interest because appearance taste characteristics possible health a liquor arise from components extracted leaf during preparation. A comprehensive study was therefore undertaken to determine contributions total soluble solids, caffeine, polyphenol contents extracts. results this show that variety, growing environment, manufacturing conditions, grade (particle size) leaves each influence final...
Increasing the size and strength of muscles represents a promising therapeutic strategy for musculoskeletal disorders, interest has focused on myostatin, negative regulator muscle growth. Various myostatin inhibitor approaches have been identified tested in models disease with varying efficacies, depending age at which inhibition occurs. Here, we describe one-time gene administration myostatin-inhibitor-proteins to enhance mass normal dystrophic mouse >2 years, even when delivered aged...
Duchenne muscular dystrophy (DMD) is a congenital X-linked myopathy caused by lack of dystrophin protein expression. In DMD, the expression many dystrophin-associated proteins (DAPs) reduced along sarcolemmal membrane, but same remain concentrated at neuromuscular junction where utrophin, homologue, expressed [Matsumura, K., Ervasti, J. M., Ohlendieck, Kahl, K. D. & Campbell, (1992) Nature (London) 360, 588–591]. This outcome has led to concept that ectopic “synaptic scaffold” DAPs and...
Objective: The purpose of this study was to explore the interrelationship between driver distraction and characteristics behavior associated with reduced highway traffic efficiency. Background: Research on three-phase theory behavioral driving suggests that a number efficient flow may be affected by distraction. Previous studies have been limited, however, fact researchers typically do not allow participants change lanes, nor they account for impact varying states performance. Methods:...
A mouse model for Duchenne muscular dystrophy engineered to produce human-like glycosylation mimics the human disease better than existing models.
ABSTRACT Agrin, a basal lamina-associated proteoglycan, is crucial nerve-derived organizer of postsynaptic differentiation at the skeletal neuromuscular junction. Because integrins serve as cellular receptors for many lamina components, we asked whether agrin interacts with integrins. Agrin-induced aggregation acetylcholine on cultured myotubes was completely blocked by antibodies to β1 integrin subunit and partially αv subunit. clustering also inhibited antisense oligonucleotides peptide...
Purpose This study evaluated the relationship of dose, plasma concentration, and time to pharmaco-dynamics zaleplon zolpidem, 2 structurally distinct benzodiazepine receptor agonists. Method Ten healthy male volunteers received single oral doses placebo, 10 mg zaleplon, 20 zolpidem in a double-blind, 5-condition crossover study, with 48 hours elapsing between trials. Plasma drug concentrations pharmacodynamic effects were measured during 8 24 after administration. Results Kinetics not...
The cytotoxic T cell (CT) GalNAc transferase, or Galgt2, is a UDP-GalNAc:beta1,4-N-acetylgalactosaminyltransferase that localized to the neuromuscular synapse in adult skeletal muscle, where it creates synaptic CT carbohydrate antigen {GalNAcbeta1,4[NeuAc(orGc)alpha2, 3]Galbeta1,4GlcNAcbeta-}. Overexpression of Galgt2 muscles transgenic mice inhibits development muscular dystrophy mdx mice, model for Duchenne dystrophy. Here, we provide physiological evidence as how may inhibit muscle...
Overexpression of GALGT2 in skeletal muscle can stimulate the glycosylation α dystroglycan and upregulation normally synaptic dystroglycan-binding proteins, some which are dystrophin laminin α2 surrogates known to be therapeutic for several forms muscular dystrophy. This article describes vascular delivery gene therapy a large animal model, rhesus macaque. Recombinant adeno-associated virus, serotype 74 (rAAVrh74), was used deliver via femoral artery gastrocnemius using an isolated focal...
Twenty-four healthy male and female subjects, who participated in this randomized, double-blind, crossover study, received single nighttime doses of zaleplon 10 mg (therapeutic dose), 20 mg, zolpidem triazolam 0.25 (positive control), placebo. Subjective behavioral ratings psychomotor tests were completed before 1.25 8.25 hours after administration the study drug. The Immediate Delayed Word Recall Digit Span Test used to assess memory. Digit-Symbol Substitution Test, Paired Associates...
The basal lamina that ensheaths skeletal muscle fibers traverses the synaptic cleft at neuromuscular junction. Synaptic and extrasynaptic portions of contain different laminin β chains: β2 (or s) synapses β1 B1) extrasynaptically. Laminin is also confined to synapselike patches on myotube surfaces in vitro, whereas present throughout extracellular matrix. This differential localization chains was analyzed by expression chimeric β1-β2 molecules cultured mouse myotubes. A 16-amino acid...
A prospective, randomized, double‐blind study to determine the postoperative efficacy of steroids in tonsillectomy was performed 49 children. single dose intravenous dexamethasone or placebo administered after each child anesthetized. Postoperatively examined for objective signs trismus (measured by interincisor distance), temperature elevation, and weight loss, as well subjective mouth odor, oral intake, pain, level activity, analgesic usage. There were no statistical differences noted any...
Utrophin is normally confined to the neuromuscular junction (NMJ) in adult muscle and partially compensates for loss of dystrophin mdx mice. We show that Akt signaling utrophin levels were diminished sarcospan (SSPN)-deficient muscle. By creating several transgenic knockout mice, we demonstrate SSPN regulates control expression. determined α-dystroglycan (α-DG) glycosylation by affecting NMJ-specific glycosyltransferase Galgt2. After cardiotoxin (CTX) injury, regenerating myofibers express...
In a phase 1/2, open-label dose escalation trial, we delivered rAAVrh74.MCK.GALGT2 (also B4GALNT2) bilaterally to the legs of two boys with Duchenne muscular dystrophy using intravascular limb infusion. Subject 1 (age 8.9 years at dosing) received 2.5 × 1013 vector genome (vg)/kg per leg (5 vg/kg total) and subject 2 6.9 5 (1 1014 total). No serious adverse events were observed. Muscle biopsy evaluated 3 or 4 months post treatment versus baseline showed evidence GALGT2 gene expression...