Publications by authors named "G A Truskey"
Objective: Juvenile dermatomyositis (JDM) involves up-regulated type I interferons (IFNs), including IFNβ, yet pathologic mechanisms remain poorly understood. We aimed to characterize the functional and structural effects of IFNβ on in vitro human pediatric myoblast growth and differentiation in a three-dimensional skeletal muscle model (myobundles).
Methods: Myobundles fabricated from myoblasts of a healthy pediatric donor were exposed to IFNβ at 0 to 5,600 IU/mL during growth (days 1-4), differentiation (days 4-11), and/or mature (days 11-18) periods.
Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disorder in children caused by a point mutation in the lamin A gene, resulting in a toxic form of lamin A called progerin. Accelerated atherosclerosis leading to heart attack and stroke are the major causes of death in these patients. Endothelial cell (EC) dysfunction contributes to the pathogenesis of HGPS related cardiovascular diseases (CVD).
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- Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare aging disorder that leads to severe heart problems and early death, typically in teenage years, due to the accumulation of a mutated protein called progerin.
- Progerin disrupts the structure of the cell nucleus, causing cellular dysfunction, inflammation, and abnormalities in gene expression, which contribute to the disease's symptoms.
- The review explores how progerin affects the mechanical functions of cells in the arteries, specifically endothelial and smooth muscle cells, emphasizing that these changes impair their ability to respond to normal blood flow and maintain proper function.
Transceptors, solute transporters that facilitate intracellular entry of molecules and also initiate intracellular signaling events, have been primarily studied in lower-order species. Ammonia, a cytotoxic endogenous metabolite, is converted to urea in hepatocytes for urinary excretion in mammals. During hyperammonemia, when hepatic metabolism is impaired, nonureagenic ammonia disposal occurs primarily in skeletal muscle.
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- * Researchers have developed a 3-D skeletal muscle model called "myobundle" to study LGMD2B, showing issues in muscle contraction, calcium management, and metabolism.
- * Treatments with specific drugs were able to improve muscle function in the model, highlighting the role of calcium leak as a key factor in the disease and the usefulness of the myobundle model for understanding LGMD2B.