Vascular Ehlers Danlos Syndrome for the Rheumatologist, Pt 2: Extra-Cellular Matrix Reloaded
In this episode we explore ways in which the extracellular matrix can be manipulated, including the story of doxycycline, TGF-beta in Marfan syndrome and whether beta blockers can reduce vascular events in vascular EDS.
- Intro 0:12
- Review of previous episode 0:28
- In this episode 2:26
- The pressure against the vessels 4:06
- The pressure against the wall 8:44
- Matrix metalloproteinases 10:16
- Tadpole study – collagen breakdown 10:35
- Tetracycline antibiotics 14:05
- Rat model – periodontal disease and hydroxyproline 14:24
- Chemically modified tetracyclines 20:14
- Mouse model – tetracycline use 22:00
- Tetracyclines and other autoimmune conditions 23:22
- Marfan syndrome 24:45
- Fibrillin and Marfan syndrome 28:48
- TGF-beta 29:36
- Mouse model – Marfan syndrome and fibrillin 31:14
- ARBs and TGF-beta 33:51
- TGF-beta and vascular EDS 37:25
- Back to the mouse model 38:38
- Protein kinase C 39:56
- Summary 40:26
Disclosures: Brown reports no relevant financial disclosures.
We’d love to hear from you! Send your comments/questions to Dr. Brown at rheuminationspodcast@healio.com. Follow us on Twitter @HRheuminations @AdamJBrownMD @HealioRheum.
References:
Bowen CJ, et al. J Clin Invest. 2020;130:686-698.
Brooke BS. Lancet. 2010;doi:10.1016/S0140-6736(10)61155-5
Dietz HC, et al. Am J Med Genet C Semin Med Genet. 2005;doi:10.1002/ajmg.c.30068.
Dubacher N, et al. Cardiovasc Res. 2020;116:457-465.
Golub LM, et al. SAGE. 1998;doi:10.1177/08959374980120010501.
Gross J, et al. PNAS. 1962;doi:10.1073/pnas.48.6.1014
Habashi JP, et al. Science. 2006;312:117-121.
Morissette R, et al. Circ Cardiovasc Genet. 2014;7:80-88.
Mullen M, et al. Lancet. 2019;394:2263-2270.
Neptune ER, et al. Nat Genet. 2003;33:407-411.
Collapse