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https://www.selleckchem.com/btk.html Pyoderma gangrenosum (PG) is a rare, inflammatory dermatologic condition characterized by painful cutaneous ulcerations. Herein, we describe the third documented case of PG arising in an elective plastic surgery patient who had undergone an otherwise uncomplicated facelift. We describe the course of her diagnosis and management of PG involving her face and neck and then progressing to her lower extremities. Although the etiology remains unknown, PG often arises in a host with another autoimmune disease. In the case described, the patient was diagnosed with an IgA gammopathy shortly after development of PG. Following the case report, the pathogenesis, diagnosis and treatment strategy of PG is briefly reviewed. Marfan syndrome is one of the most common inherited disorders of connective tissue caused by fibrillin-1 mutations, characterized by enhanced transcription factor AP-1 DNA binding activity and subsequently abnormally increased expression and activity of matrix-metalloproteinases (MMPs). We aimed to establish a novel adeno-associated virus (AAV)-based strategy for long-term expression of an AP-1 neutralising RNA hairpin (hp) decoy oligonucleotide (dON) in the aorta to prevent aortic elastolysis in a murine model of Marfan syndrome. Using fibrillin-1 hypomorphic mice (mgR/mgR), aortic grafts from young (9 weeks old) donor mgR/mgR mice were transduced ex vivo with AAV vectors and implanted as infrarenal aortic interposition grafts in mgR/mgR mice. Grafts were explanted after 30 days. For in vitro studies isolated primary aortic smooth muscle cells from mgR/mgR mice were used. Elastica-van-Giesson staining visualized elastolysis, ROS production was assessed using DHE staining. RNA F.I.S.H. verified AP-1 hp dOial to prevent life-threatening elastolysis and aortic complications. This study provides a novel single treatment option to achieve long-term expression of a transcription factor AP-1 neutralising decoy oligonucleotide in t
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