Osteogenesis imperfecta (OI), a heritable disease caused by molecular defects in type I collagen, is characterized by skeletal deformities and brittle bones. The heterozygous and homozygous oim mice (oim/+ and oim/oim) exhibit mild and severe OI phenotypes, respectively, serving as controlled animal models of this disease. In the current study, bone geometry, mechanics, and material properties of 1-year-old mice were evaluated to determine factors that influence the severity of phenotype in OI. The oim/oim mice exhibited significantly smaller body size, femur length, and moment of area compared with oim/+ and wild-type (+/+) controls. The oim/oim femur mechanical properties of failure torque and stiffness were 40% and 30%, respectively, of the +/+ values, and 53% and 36% of the oim/+ values. Collagen content was reduced by 20% in the oim/oim compared with +/+ bone and tended to be intermediate to these values for the oim/+. Mineral content was not significantly different between the oim/oim and +/+ bones. However, the oim/oim ash content was significantly reduced compared with that of the oim/+. Mineral carbonate content was reduced by 23% in the oim/oim bone compared with controls. Mineral crystallinity was reduced in the oim/oim and oim/+ bone compared with controls. Overall, for the majority of parameters examined (geometrical, mechanical, and material), the oim/+ values were intermediate to those of the oim/oim and +/+, a finding that parallels the phenotypes of the mice. This provides evidence that specific material properties, such as mineral crystallinity and collagen content, are indicative and possibly predictive of bone fragility in this mouse model, and by analogy in human OI.
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http://dx.doi.org/10.1359/jbmr.1999.14.2.264 | DOI Listing |
Circ Heart Fail
January 2025
Aswan Heart Center, Magdi Yacoub Heart Foundation, Egypt (A.M.I., M.R., A. Elsawy, M.H., S.H., W.E., A. Elaithy, A. Elguindy, A. Afifi, Y.A., M.Y.).
Background: Changes in the phenotype and genotype in hypertrophic cardiomyopathy (HCM) are thought to involve the myocardium as well as extracardiac tissues. Here, we describe the structural and functional changes in the ascending aorta of obstructive patients with HCM.
Methods: Changes in the aortic wall were studied in a cohort of 101 consecutive patients with HCM undergoing myectomy and 9 normal controls.
Rev Med Suisse
January 2025
Service de néphrologie, Département de médecine, Hôpitaux universitaires de Genève, Genève 14.
Certain molecules, such as GLP-1 agonists and endothelin antagonists, possess nephroprotective properties. When treating IgA nephropathy, endothelin antagonists and sibeprenlimab have shown effectiveness in slowing the progression of chronic kidney isease. Additionally, the infusion of amino acids can reduce the incidence of mild acute kidney injury following cardiac surgery.
View Article and Find Full Text PDFEur Heart J Digit Health
January 2025
Division of Cardiovascular Medicine, Department of Clinical Sciences, Danderyd Hospital, Karolinska Institutet, SE-182 88 Stockholm, Sweden.
Aims: A simplified version of the history, electrocardiogram, age, risk factors, troponin (HEART) score, excluding troponin, has been proposed to rule-out major adverse cardiac events (MACEs). Computerized history taking (CHT) provides a systematic and automated method to obtain information necessary to calculate the HEAR score. We aimed to evaluate the efficacy and diagnostic accuracy of CHT in calculating the HEAR score for predicting MACE.
View Article and Find Full Text PDFFront Nutr
January 2025
College of Food Science, Northeast Agricultural University, Harbin, Heilongjiang, China.
Introduction: Frozen pork can reduce the quality of the meat and alter the digestibility and bioavailability of meat proteins in the human body. In this study, we investigated the changes in the basic composition during frozen storage and their effects on the structural properties of digestion products after protein digestion.
Methods: The impacts of frozen storage at different temperatures (-8, -18, -25, and -40°C) and for different times (1, 3, 6, 9, and 12 months) on the basic components and digestive characteristics of pork were evaluated.
Front Genome Ed
January 2025
State Key Laboratory of Animal Biotech Breeding, China Agricultural University, Beijing, China.
Primordial germ cells (PGCs) play a crucial role in transmitting genetic information to the next-generation. In chickens, genetically edited PGCs can be propagated and subsequently transplanted into recipient embryos to produce offspring with desired genetic traits. However, during early embryogenesis, the effects of external conditions on PGC migration through the vascular system to the gonads have yet to be explored, which may affect the efficiency of preparing gene-edited chickens.
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