Implementation of Drug‐Induced Rhabdomyolysis and Acute Kidney Injury in Microphysiological System
A modular Muscle–Kidney proximal tubule‐on‐a‐chip integrates 3D skeletal muscle and renal proximal tubule tissues to model drug‐induced rhabdomyolysis and acute kidney injury. The coculture system enables dynamic tissue interaction, functional contraction monitoring, and quantification of nephrotoxicity, revealing drug side effect‐induced metabolic ...
Jaesang Kim +4 more
wiley +1 more source
Gram‐Scale Synthesis of an Arylsulfonamide‐Type Alkaline Phosphatase Inhibitor
A larger amount of a tissue‐nonspecific alkaline phosphatase inhibitor, 5‐((5‐chloro‐2‐methoxyphenyl)sulfonamido)nicotinamide, was synthesized in five steps using a parallel method without the need for chromatography or crystallization. Because a single batch was used for in vivo studies in a large population of mice, the pharmacological results were ...
Zsolt Rapi +2 more
wiley +1 more source
Inhibition of Tissue-Nonspecific Alkaline Phosphatase Attenuates Ectopic Mineralization in the Abcc6
Pseudoxanthoma elasticum (PXE), a prototype of heritable ectopic mineralization disorders, is caused by mutations in the ABCC6 gene encoding a putative efflux transporter ABCC6.
Millan, Jose Luis +15 more
core +1 more source
Pseudoxanthoma elasticum: cardiac findings in patients and Abcc6-deficient mouse model.
BackgroundPseudoxanthoma elasticum (PXE), caused by mutations in the ABCC6 gene, is a rare multiorgan disease characterized by the mineralization and fragmentation of elastic fibers in connective tissue.
Fabrice Prunier +11 more
doaj +1 more source
Rate of change of carotid intima-media thickness with magnesium administration in Abcc6⁻/⁻ mice. [PDF]
Pseudoxanthoma elasticum (PXE), caused by mutations in the ABCC6 gene, demonstrates progressive build-up of calcium phosphate and proteoglycans in the skin, eye, and arteries, and is associated to myocardial infarctions, stroke, blindness, and elevated ...
Fred Rincon +5 more
core +1 more source
Untersuchung der Auswirkungen einer Abcc6-Defizienz auf die Cholesterinhomöostase im Mausmodell [PDF]
Ibold B. Untersuchung der Auswirkungen einer Abcc6-Defizienz auf die Cholesterinhomöostase im Mausmodell. Bielefeld: Universität Bielefeld; 2018.Sequenzvariationen des Adenosintriphosphat-Bindungskassetten (ABC)-Transporter der Unterfamilie C, Mitglied ...
Ibold, Bettina
core
Targeted ablation of the abcc6 gene results in ectopic mineralization of connective tissues.
Pseudoxanthoma elasticum (PXE), characterized by connective tissue mineralization of the skin, eyes, and cardiovascular system, is caused by mutations in the ABCC6 gene.
Choi, H Y +10 more
core +1 more source
A mouse model of beta-thalassemia shows a liver-specific down-regulation of Abcc6 expression [PDF]
beta-Thalassemia and pseudoxanthoma elasticum (PXE) are distinct genetic disorders. Yet, a dystrophic mineralization phenotype similar to PXE has frequently been associated with beta-thalassemia or sickle cell anemia patients of Mediterranean descent ...
V. Douet +14 more
core +1 more source
Thalassemia, once associated with limited survival, now sees extended life expectancy due to treatment advancements, but new complications such as pseudoxanthoma elasticum (PXE)-like syndrome are emerging.
Marialuisa Zedde, Rosario Pascarella
doaj +1 more source
Expression and in vivo rescue of human ABCC6 disease-causing mutants in mouse liver.
Loss-of-function mutations in ABCC6 can cause chronic or acute forms of dystrophic mineralization described in disease models such as pseudoxanthoma elasticum (OMIM 26480) in human and dystrophic cardiac calcification in mice.
Olivier Le Saux +9 more
doaj +1 more source

