Document Detail


Altered tissue behavior of a non-aneurysmal descending thoracic aorta in the mouse model of Marfan syndrome.
MedLine Citation:
PMID:  22105919     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Aortic aneurysm is predominantly found in the ascending aorta in patients with Marfan syndrome (MFS). However, descending aortic disease has emerged as a problem since people are living longer because of improved medical and surgical management of the ascending aorta. Diagnostic procedures before disease onset and the mechanisms involved in the transition of normal aortic tissue to aneurysm remain unclear. We determined signs of descending aortic disease before disease onset in mice with a mutation in the fibrillin 1 gene (Fbn1 (+/C1039G)), a validated mouse model of disease susceptibility and progression of aortic aneurysm of MFS. We analyzed a tubular unfixed non-aneurysmal descending thoracic aorta from 8-month-old wild-type and Fbn1 (+/C1039G) mice by a tubular biaxial tester that works in conjunction with a two-photon nonlinear microscope. Fbn1 (+/C1039G) mouse aorta was more compliant in the circumferential direction. Two-photon imaging showed defective organization of adventitial collagen fibers in the pressurized aortas of Fbn1 (+/C1039G) mice. Moreover, disruption in the elastic lamina was noted in the absence of aneurysms in pressurized aortas but not unpressurized aortas of Fbn1 (+/C1039G) mice. At the molecular level, this altered tissue behavior in non-aneurysmal descending aortas of Fbn1 (+/C1039G) mice was accompanied by an increasing trend of canonical but not noncanonical, transforming growth factor-β (TGFβ) signaling. Finally, assays of in vitro collagen lattice formation in mouse wild-type and TGFβ1-deficient embryonic fibroblasts indicate that TGFβ1 can regulate collagen organization. The ability to reveal the presence of altered biomechanics and microstructure coupled with subtle changes in TGFβ signaling provides a novel surrogate measure of tissue susceptibility to aneurysm before disease onset.
Authors:
Darren Haskett; Jefferson J Doyle; Connie Gard; Hwudaurw Chen; Corbie Ball; Mark A Estabrook; Alejandra C Encinas; Harry C Dietz; Urs Utzinger; Jonathan P Vande Geest; Mohamad Azhar
Related Documents :
10148969 - Balloon dilatation for discrete subaortic stenosis: immediate and intermediate-term res...
2932769 - Percutaneous transluminal angioplasty in the region of the aortic bifurcation. the two-...
10931629 - Vasoseal after intra-aortic balloon pump removal: a pilot study.
10921729 - Intraoperative balloon angioplasty for aortic coarctation after norwood operation.
10362369 - Balloon dilatation for critical pulmonary stenosis.
1970619 - Morphological changes in normal canine basilar arteries after transluminal angioplasty.
22578699 - How to perform combined cutting balloon and high pressure balloon valvuloplasty for dog...
18555239 - Human testicular arterial supply: gross anatomy, corrosion cast, and radiologic study.
2014849 - Pulmonary injury associated with antepartum pyelonephritis: can patients at risk be ide...
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-11-22
Journal Detail:
Title:  Cell and tissue research     Volume:  -     ISSN:  1432-0878     ISO Abbreviation:  -     Publication Date:  2011 Nov 
Date Detail:
Created Date:  2011-11-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0417625     Medline TA:  Cell Tissue Res     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Graduate Interdisciplinary Program of Biomedical Engineering, University of Arizona, Tucson, Ariz., USA.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Altholactone Induces Apoptotic Cell Death in Human Colorectal Cancer Cells.
Next Document:  Comparison of the osteogenic potentials of autologous cultured osteoblasts and mesenchymal stem cell...