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Heterozygous missense mutation of the fibrinogen gene associated with cryptogenic liver disease in a 15-months-old Canadian caucasian child.
Ultrastruct Pathol
December 2024
Anatomical Pathology, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.
Hepatic fibrinogen storage disease is an uncommon autosomal dominant hereditary illness marked by hypofibrinogenemia and the accumulation of variant fibrinogen in the hepatic endoplasmic reticulum. We present an asymptomatic 15-month-old male with elevated liver enzymes. Test results indicate hypofibrinogenemia.
View Article and Find Full Text PDFLoss of hepatocyte Usp53 protects mice from a form of xenobiotic-induced liver injury.
Biochim Biophys Acta Mol Basis Dis
December 2024
The Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai 201102, China; Shanghai Key Laboratory of Birth Defect, Shanghai 201102, China. Electronic address:
Background: Ubiquitin-specific protease 53 (USP53) deficiency is associated with familial intrahepatic cholestasis in which serum gamma-glutamyl transferase (GGT) activity is relatively low. However, how USP53 deficiency contributes to cholestasis is obscure. No animal model has been reported.
View Article and Find Full Text PDFPlastic additives affect estrogenic pathways and lipid metabolism in precision - cut - liver slices in Atlantic cod (Gadus morhua).
Sci Total Environ
January 2025
Department of Paraclinical Sciences, Norwegian University of Life Sciences, Oslo, Norway.
The overall aim of the present study was to determine if exposure to three high volume plastic additives, including diethylhexyl phthalate (DEHP), bisphenol A (BPA) and benzotriazoles (BT), have the potential to promote adverse effects in Atlantic cod (G. morhua). Ex vivo precision cut - liver slices (PCLS) from six male juvenile Atlantic cod were exposed to four concentrations of mono-(2-ethylhexyl)-phthalate (MEHP, the main metabolite of DEHP), BPA and BT both singly and in mixtures ranging from 0.
View Article and Find Full Text PDFExosomes Derived from Adipose Mesenhymal Stem Cells Ameliorate Lipid Metabolism Disturbances Following Liver Ischemia-Reperfusion Injury in Miniature Swine.
Int J Mol Sci
December 2024
College of Veterinary Medicine, Northeast Agricultural University, Harbin 150030, China.
The liver plays a crucial role in regulating lipid metabolism. Our study examined the impact of Exosomes derived from adipose mesenchymal stem cells (ADSCs-Exo) on lipid metabolism following liver ischemia-reperfusion injury (IRI) combined with partial hepatectomy. We developed a miniature swine model for a minimally invasive hemi-hepatectomy combined with liver IRI.
View Article and Find Full Text PDFMetformin ameliorates diabetes-induced hepatic ultrastructural damage and the immune biomarker CD86 and inflammation in rats.
Ultrastruct Pathol
January 2025
Department of Family and Community Medicine, College of Medicine, Princess Nourah bint Abdulrahman University, Riyadh, Saudi Arabia.
Diabetes is a known inducer of hepatic ultrastructural alterations, and the expression of the immune biomarker that involves in T-cell immunity, cluster of differentiation 86 (CD86) is increased in diabetic patients with liver cirrhosis. The antidiabetic drug metformin has not previously been used to protect against type 2 diabetes mellitus (T2DM)-induced alternations in hepatic ultrastructure and the induction of the hepatic CD86/inflammation axis in diabetic animal models induced by streptozotocin and a high fat diet. To test our hypotheses, T2DM was induced in rats (model group) and the protective animals were treated with the antidiabetic drug metformin (200 mg/kg) until being sacrificed at week 12.
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