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Healio Rheuminations

Vascular Ehlers Danlos Syndrome for the Rheumatologist, Pt 2: Extra-Cellular Matrix Reloaded

Feb 28, 2023
41:57

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.

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