A5071657615- Connective tissue disorders research
- Eicosanoids and Hypertension Pharmacology
- Aortic aneurysm repair treatments
- Nitric Oxide and Endothelin Effects
- Hip disorders and treatments
- Peptidase Inhibition and Analysis
- Mitochondrial Function and Pathology
- Protease and Inhibitor Mechanisms
Charité - Universitätsmedizin Berlin
2019
Humboldt-Universität zu Berlin
2019
Freie Universität Berlin
2019
Abstract Marfan syndrome ( MFS ) is an autosomal dominant genetic disorder caused by mutations in the FBN 1 gene. Although many peripheral tissues are affected, aortic complications, such as dilation, dissection and rupture, leading causes of ‐related mortality. Aberrant TGF ‐beta signalling plays a major role pathophysiology . However, contributing mechanisms still poorly understood. Here, we aimed at identifying novel aorta‐specific pathways involved For this purpose, employed Fbn1...
Defects in the extracellular matrix protein fibrillin-1 that perturb transforming growth factor beta (TGFβ) bioavailability lead to Marfan syndrome (MFS). MFS is an autosomal-dominant disorder, which associated with connective tissue and skeletal defects, among others. To date, it unclear how biological sex impacts structural functional properties of bone MFS. The aim this study was investigate effects on microarchitecture mechanical mice deficient fibrillin-1, a model human Bones...
Dichloroacetate (DCA) is a small, orally available inhibitor of pyruvate dehydrogenase kinase (PDK). Consequently, DCA stimulates the activity complex (PDC), leading to glucose oxidation in mitochondria. Therapeutically, employed decrease lactate production and has been used acute treatment certain metabolic diseases, such as lactic acidosis. More recently, reported have anti‐cancer efficacy suggested chemotherapeutic. However, therapeutic agent may adverse effects, it cause hepatocellular...