A5075197863- Dermatological and Skeletal Disorders
- Heterotopic Ossification and Related Conditions
- Connective tissue disorders research
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Bone Metabolism and Diseases
- Kidney Stones and Urolithiasis Treatments
- Hypertrophic osteoarthropathy and related conditions
- Cardiomyopathy and Myosin Studies
- Inflammatory Myopathies and Dermatomyositis
- Cardiovascular Function and Risk Factors
- Cellular Mechanics and Interactions
- Alkaline Phosphatase Research Studies
University of Hawaiʻi at Mānoa
2017-2024
University of Hawaii System
2021
Background Pseudoxanthoma elasticum (PXE) is a genetic disease caused by mutations in the ABCC6 gene that result low pyrophosphate levels and subsequent progressive soft tissue calcifications. PXE mainly affects skin, retina, arteries. However, many patients with experience kidney stones. We determined prevalence of this pathology examined possible underlying mechanisms murine models. Methods conducted retrospective study large cohort analyzed urine samples kidneys from Abcc6 −/− mice at...
Abstract ABCC6 deficiency promotes ectopic calcification; however, circumstantial evidence suggested that may also influence atherosclerosis. The present study addressed the role of in atherosclerosis using Ldlr −/− mice and pseudoxanthoma elasticum (PXE) patients. Mice lacking Abcc6 genes were fed an atherogenic diet for 16 weeks before intimal calcification, aortic plaque formation lipoprotein profile evaluated. Cholesterol efflux expression several inflammation, cholesterol...
Abstract Supraphysiological levels of the osteoblast‐enriched mineralization regulator ectonucleotide pyrophosphatase or phosphodiesterase‐1 (NPP1) is associated with type 2 diabetes mellitus. We determined impact osteoblast‐specific Enpp1 ablation on skeletal structure and metabolic phenotype in mice. Female, but not male, 6‐week‐old mice lacking osteoblast NPP1 expression (osteoblast‐specific knockout [KO]) exhibited increased femoral bone volume total (17.50% vs. 11.67%; p < .01),...
Increased myocardial glycolysis is protective against ischemic injury. Glucose uptake via glucose transporter GLUT4 the rate-limiting step of in cardiomyocytes. delivery to cardiomyocyte membrane can be triggered by insulin or increased ATP-demand (i.e. during ischemia) AMP-activated protein kinase (AMPK). Previous studies adipocytes and our recent study skeletal muscle demonstrated that exocyst trafficking complex critical for exocytosis response insulin. But it not known if insulin-insulin...