Targeting TGF-beta and the extracellular matrix in Marfan's syndrome

Raghu Kalluri; Yuchi Han

doi:10.1016/j.devcel.2008.06.005

Targeting TGF-beta and the extracellular matrix in Marfan's syndrome

Dev Cell. 2008 Jul;15(1):1-2. doi: 10.1016/j.devcel.2008.06.005.

Authors

Raghu Kalluri¹, Yuchi Han

Affiliation

¹ Division of Matrix Biology, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA. rkalluri@bidmc.harvard.edu

PMID: 18606132
DOI: 10.1016/j.devcel.2008.06.005

Abstract

Marfan's syndrome is a genetic disorder affecting connective tissues, and it can lead to death due to aortic defects if left untreated. beta-blocker therapy has been used to slow the progression of this disease. Brooke et al. (2008) now report that combining angiotensin II receptor blockade by losartan with beta-blocker treatment is an effective treatment combination therapy for this disorder.

MeSH terms

Angiotensin II Type 1 Receptor Blockers / antagonists & inhibitors
Angiotensin II Type 1 Receptor Blockers / therapeutic use*
Child
Disease Progression
Extracellular Matrix / chemistry*
Extracellular Matrix / genetics
Fibrillins
Genes, Dominant
Humans
Losartan / therapeutic use*
Marfan Syndrome / diagnostic imaging
Marfan Syndrome / drug therapy*
Marfan Syndrome / genetics
Marfan Syndrome / pathology
Microfilament Proteins / genetics
Mutation
Radiography
Signal Transduction / drug effects
Transforming Growth Factor beta / antagonists & inhibitors*

Substances

Angiotensin II Type 1 Receptor Blockers
Fibrillins
Microfilament Proteins
Transforming Growth Factor beta
Losartan