A5017530763- Connective tissue disorders research
- Aortic Disease and Treatment Approaches
- Aortic aneurysm repair treatments
- TGF-β signaling in diseases
- Congenital heart defects research
- Bone fractures and treatments
- Peptidase Inhibition and Analysis
- Spine and Intervertebral Disc Pathology
- Protease and Inhibitor Mechanisms
- Orthopaedic implants and arthroplasty
- Signaling Pathways in Disease
- Infectious Aortic and Vascular Conditions
- Connective Tissue Growth Factor Research
UNSW Sydney
2024
Cedars-Sinai Medical Center
2024
Locate Bio (United Kingdom)
2024
University Hospital Cologne
2021-2022
University of Cologne
2021-2022
Since their discovery as pluripotent cytokines extractable from bone matrix, it has been speculated how morphogenetic proteins (BMPs) become released and activated the extracellular matrix (ECM). In contrast to TGF-βs, most investigated BMPs are secreted bioactive prodomain (PD)–growth factor (GF) complexes (CPLXs). Recently, we demonstrated that PD-dependent targeting of BMP-7 CPLXs fibrillin microfibril (FMF) components fibrillin-1 -2 represents a BMP sequestration mechanism by rendering...
Abstract Fibrillin-1 assembles into microfibrils that not only define the structural integrity and biomechanics of aorta but also target sequester growth factors within extracellular microenvironment aortic resident cells. To better understand how dominant negative effects on fibrillin microfibril stability manifest in factor driven disease, we analyzed early events aneurysm formation first two weeks postnatal life Fbn1 GT8 Marfan mouse model. Echocardiography analysis homozygous mice showed...
Abstract Marfan syndrome (MFS) is the most prevalent inherited connective tissue disorder, still remains uncurable, and characterized by high mortality at early age driven dissection rupture of thoracic aortic aneurysms. MFS caused mutations in fibrillin-1 gene aberrant TGFß signaling. Here we addressed whether myeloperoxidase (MPO), a leukocyte derived enzyme with potent matrix modulating properties also influences phenotype MFS. patients displayed increased circulating MPO levels compared...
Abstract Abdominal aortic aneurysms (AAA) are a common vascular disorder with high mortality due to the prevalence of ruptures. The underlying pathomechanisms complex and involve immune cell infiltration degradation extracellular matrix (ECM). Hyaluronan (HA), synthesized at plasma membrane by three HA synthase isoenzymes (HAS1-3), is not only major constituent ECM but also known directly affect phenotype smooth muscle cells as well immunological responses. Specifically, HAS3 isoenzyme has...