A5007584613- Muscle Physiology and Disorders
- Cardiomyopathy and Myosin Studies
- melanin and skin pigmentation
- Adipose Tissue and Metabolism
- Tissue Engineering and Regenerative Medicine
- Biochemical Analysis and Sensing Techniques
- Neuropeptides and Animal Physiology
- Cardiovascular Function and Risk Factors
- Cardiovascular Effects of Exercise
- Advanced Sensor and Energy Harvesting Materials
- Electrospun Nanofibers in Biomedical Applications
- Mitochondrial Function and Pathology
- Genetic Neurodegenerative Diseases
- Skin Protection and Aging
- Gastrointestinal motility and disorders
- Gout, Hyperuricemia, Uric Acid
- RNA regulation and disease
- Neurobiology and Insect Physiology Research
- Neuroscience and Neuropharmacology Research
- Muscle metabolism and nutrition
- Viral Infections and Immunology Research
- Glycosylation and Glycoproteins Research
- Exercise and Physiological Responses
- Pharmacological Receptor Mechanisms and Effects
- Cannabis and Cannabinoid Research
University of Minnesota Medical Center
2011-2024
Minneapolis Heart Institute Foundation
2010-2024
University of Minnesota
2011-2024
Northumbria Healthcare NHS Foundation Trust
2024
University of Michigan–Ann Arbor
2006-2008
Laboratoire d’immunologie intégrative du cancer
2005
University of Wisconsin–Madison
1991
Twin Cities Orthopedics
1990
Future Science Group (United Kingdom)
1989
Utrecht University
1973
Duchenne muscular dystrophy (DMD) is the most common, lethal genetic disorder of children.A number animal models exist, but effective model for characterizing structural and functional properties dystrophin therapeutic interventions has been mdx mouse.Despite ϳ ϳ20 years investigations mouse, impact disease on life span mice cause death remain unresolved.Consequently, a study mouse was designed that included cohorts male female wild-type C57BL/10 housed under specific pathogen-free...
Rationale: One goal of cardiac tissue engineering is the generation a living, human pump in vitro that could replace animal models and eventually serve as an vivo therapeutic. Models replicate geometrically complex structure heart, harboring chambers large vessels with soft biomaterials, can be achieved using 3-dimensional bioprinting. Yet, inclusion contiguous, living muscle to support function has not been achieved. This largely due challenge attaining high densities cardiomyocytes—a...
Duchenne muscular dystrophy (DMD) is a fatal disease of striated muscle deterioration resulting from the loss cytoskeletal protein dystrophin. Most patients develop significant cardiomyopathy, with heart failure being second leading cause death in DMD. Compared extensive studies on skeletal defects and potential therapy DMD, very little attention has been directed at increasing incidence Here we show that single systemic injection recombinant adeno-associated virus (rAAV2/6) harboring...
Duchenne muscular dystrophy (DMD) is a fatal disease characterized by deterioration of striated muscle, affecting skeletal and cardiac muscles. Recently, several therapeutic approaches have shown promise for repairing dystrophic However, these methods often leave the heart untreated. Here we show that, in comparison to fully dystrophin-deficient animals, targeted transgenic repair but not otherwise (mdx) mice paradoxically elicited fivefold increase injury dilated cardiomyopathy animals...
Duchenne muscular dystrophy (DMD) is a fatal disease of striated muscle deterioration caused by lack the cytoskeletal protein dystrophin. Dystrophin deficiency causes membrane instability, skeletal wasting, cardiomyopathy, and heart failure. Advances in palliative respiratory care have increased incidence DMD patients, for which there no cure or effective therapy. Here we shown that chronic infusion membrane-sealing poloxamer to severely affected dystrophic dogs reduced myocardial fibrosis,...
ADVERTISEMENT RETURN TO ISSUEPREVArticleNEXTFunction of dopachrome oxidoreductase and metal ions in conversion the eumelanin pathwayLaura J. Leonard, DeWayne Townsend, Richard A. KingCite this: Biochemistry 1988, 27, 16, 6156–6159Publication Date (Print):August 1, 1988Publication History Published online1 May 2002Published inissue 1 August 1988https://pubs.acs.org/doi/10.1021/bi00416a049https://doi.org/10.1021/bi00416a049research-articleACS PublicationsRequest reuse permissionsArticle...
Several types of autosomal recessive oculocutaneous albinism (OCA) are associated with abnormal tyrosinase function and a generalized reduction in or absence cutaneous eye melanin. Each is thought to result from different mutant allele at the locus, mutation producing an enzyme little no activity all involved tissues. In this paper, we report new type OCA that results temperature-sensitive enzyme. The proband had white hair warmer areas (scalp axilla) progressively darker cooler...
Duchenne muscular dystrophy (DMD) and limb girdle (LGMD) 2C-F result from the loss of dystrophin sarcoglycans, respectively. Dystrophin, a cytoskeletal protein, is closely associated with membrane-bound sarcoglycan complex. Despite this tight biochemical association, function subunits may differ. The in skeletal muscle results that highly susceptible to contraction-induced damage, but mice lacking γ- or δ-sarcoglycan are less susceptible. Using mouse models DMD, LGMD-2C, LGMD-2F, we...
Excessive and/or persistent activation of calcium-calmodulin protein kinase II (CaMKII) is detrimental in acute and chronic cardiac injury. However, intrinsic regulators CaMKII activity are poorly understood. We find that cellular retinoic acid-binding 1 (CRABP1) directly interacts with uncover a functional role for CRABP1 regulating activation. generated Crabp1-null mice (CKO) C57BL/6J background pathophysiological studies. CKO develop hypertrophy as adults, exhibiting significant left...
Abstract Duchenne muscular dystrophy (DMD) is a disease marked by the development of skeletal muscle weakness and wasting. DMD results from mutations in gene for cytoskeletal protein dystrophin. The loss dystrophin expression not limited to but has multiple systemic consequences. Managing nutritional requirements an important aspect clinical care patients complicated poor understanding role dystrophin, dystrophic processes, regulating metabolism. Here, we show that mdx mice, genetic model...
Heart failure with preserved ejection fraction (HFpEF) is a complex clinical syndrome, but predominant subset of HFpEF patients has metabolic syndrome (MetS). Mechanistically, systemic, nonresolving inflammation associated MetS might drive remodeling. Free fatty acid receptor 4 (Ffar4) GPCR for long-chain acids that attenuates dysfunction and resolves inflammation. Therefore, we hypothesized Ffar4 would attenuate remodeling in secondary to (HFpEF-MetS). To test this hypothesis, mice systemic...
Abstract The distribution and kinetic parameters of tyrosinase (EC 1.14.–18.1) from anagen hair follicles the C57BL/6J mouse several c ‐locus mutants were investigated. results showed that mutations not only influenced total activity, but subcellular as well. by pigment mutations, suggesting influencing structure enzyme.
Duchenne muscular dystrophy (DMD) is a progressive disease of striated muscle deterioration. Respiratory and cardiac dysfunction are particularly clinically relevant because they result in the leading causes death DMD patients. Despite clinical physiological significance these systems, little has been done to understand cardiorespiratory interaction DMD. We show here that prior onset global dysfunction, dystrophin-deficient mdx mice have increased fibrosis with right ventricle being...
Abstract Objective To determine whether polysaccharide storage myopathy (PSSM) in Quarter Horses is attributable to a defect glycolysis or the allosteric regulation of phosphofructokinase (PFK) enzyme. Animals Muscle biopsy specimens were obtained from 6 with PSSM and 8 Horse Thoroughbred control horses. Procedures Maximal activity glycogenolytic glycolytic enzymes was determined spectrophotometrically. PFK for each horse at pH 8.0, 7.0 when variable concentrations activators, fructose...