A5069693530- Muscle Physiology and Disorders
- Tissue Engineering and Regenerative Medicine
- Congenital heart defects research
- Virus-based gene therapy research
- Cardiomyopathy and Myosin Studies
- CRISPR and Genetic Engineering
- Genetics, Aging, and Longevity in Model Organisms
- Muscle metabolism and nutrition
- Muscle activation and electromyography studies
- Effects of Environmental Stressors on Livestock
- Neurogenetic and Muscular Disorders Research
- Bone fractures and treatments
- Adipose Tissue and Metabolism
- Viral Infectious Diseases and Gene Expression in Insects
- Exercise and Physiological Responses
- Animal Behavior and Welfare Studies
- Respiratory Support and Mechanisms
- Animal Nutrition and Physiology
- Silk-based biomaterials and applications
Texas A&M University
2015-2024
University of North Carolina at Chapel Hill
2018
Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease caused by the absence of dystrophin, membrane-stabilizing protein encoded DMD gene. Although mouse models provide insight into potential corrective therapy, data from genetically homologous large animals, such as dystrophin-deficient golden retriever (GRMD) model, may more readily translate to humans. To evaluate clinical translatability an adeno-associated virus serotype 9 vector (AAV9)–microdystrophin (μDys5)...
Duchenne muscular dystrophy (DMD) is caused by mutations in the DMD gene that abolish expression of dystrophin protein. Dogs with genetic homologue, golden retriever dog (GRMD), have a splice site mutation leads to skipping exon 7 and stop codon transcript. Gene editing via homology-directed repair (HDR) has been used mdx mouse model but not GRMD. In this study, we clustered regularly interspaced short palindromic repeats (CRISPR) transcription activator-like effector nucleases (TALEN)...
Background Duchenne muscular dystrophy ( DMD ) is an X‐linked disease that causes progressive muscle weakness. Affected boys typically die from respiratory or cardiac failure. Golden retriever GRMD genetically homologous with and analogous skeletal disease. Previous studies have detailed features of cardiomyopathy in mostly young dogs. Cardiac not well characterized adult dogs, magnetic resonance CMR imaging been completed. Methods Results We evaluated echocardiography 24 dogs at different...
Metabolic dysfunction in Duchenne muscular dystrophy (DMD) is characterized by reduced glycolytic and oxidative enzymes, decreased abnormal mitochondria, ATP, increased stress. We analyzed glucose metabolism as a potential disease biomarker the genetically homologous golden retriever (GRMD) dog with molecular, biochemical, vivo imaging. Pelvic limb skeletal muscle left ventricle tissue from heart were mRNA profiling, qPCR, western blotting, immunofluorescence microscopy for primary...
Dystrophin is a key cytoskeletal protein coded by the Duchenne muscular dystrophy (DMD) gene located on X-chromosome. Truncating mutations in DMD cause loss of dystrophin and classical clinical syndrome. Spontaneous associated phenotypes occur several other species. The mdx mouse model golden retriever (GRMD) canine have been used extensively to study disease pathogenesis show efficacy side effects putative treatments. Certain high-risk, so-called hot spot areas can be particularly helpful...
We have examined the effects of intravenous (IV) delivery rAAVrh74.MHCK7. GALGT2 in golden retriever muscular dystrophy (GRMD) model Duchenne Muscular Dystrophy (DMD). After baseline testing, GRMD dogs were treated at 3 months age and reassessed 6 months. This 3–6 month range is a period rapid disease progression, thus offering relatively short window to establish treatment efficacy. Measures analyzed included muscle AAV transduction, transgene expression, -induced glycosylation, pathology,...
Duchenne muscular dystrophy (DMD) is a lethal, X-chromosome linked muscle-wasting disease affecting about 1 in 3500–6000 boys worldwide. Myofibre necrosis and subsequent loss of muscle mass are due to several molecular sequelae, such as inflammation oxidative stress. We have recently shown increased neutrophils, highly reactive oxidant hypochlorous acid (HOCl) generation by myeloperoxidase (MPO), associated stress from the GRMD dog mdx mouse models for DMD. These findings led us hypothesise...
Viscoelastic Response (VisR) ultrasound is a new Acoustic Radiation Force (ARF)-based imaging method that uses two successive ARF excitations, delivered to the same region of excitation, approximate creep response in tissue and thereby estimate viscoelastic property. The viscoelasticity dystrophic muscle altered over time by ongoing necrotic, fatty, fibrous degenerative changes. Evaluating such changes VisR Golden Retriever Muscular Dystrophy (GRMD) canine model, with comparison matched MRI...
Abstract Background Duchenne muscular dystrophy (DMD) and genetically homologous golden retriever (GRMD) are X-linked conditions causing progressive muscle wasting cardiomyopathy. We previously defined a DMD-like dilated cardiomyopathy in adult GRMD dogs. The goal of this study was to extend our work characterize the early natural history cardiac stress response young Methods Age-matched (N=7), carrier (N=10), normal (N=8) littermates at 3, 6, 12 months age were prospectively enrolled....
In Golden Retriever Muscular Dystrophy (GRMD) dogs, a relevant animal model of human Duchenne muscular dystrophy (DMD), skeletal muscles progressively inflame, necrose, and undergo fibrous fatty deposition. These degenerative changes alter the composition structure muscle, which affects degree directional variation, or anisotropy, in tissue mechanical properties. Further, such spatial distribution property across muscles. this work we investigate relevance anisotropy texture, estimated using...
Introduction: Duchenne muscular dystrophy (DMD) and the genetically homologous model, golden retriever (GRMD), are x-linked conditions that cause progressive muscle wasting cardiomyopathy. We previously defined a late onset DMD-like dilated cardiomyopathy in adult GRMD dogs aimed to extend work young used for preclinical studies. The goal of this study was characterize early natural history cardiac stress response Methods: A prospective imaging completed 10 8 age-matched normal littermates...