Journal
PHYSIOLOGICAL GENOMICS
Volume 19, Issue 2, Pages 151-154Publisher
AMER PHYSIOLOGICAL SOC
DOI: 10.1152/physiolgenomics.00092.2004
Keywords
elastic fiber; growth factor signaling; Marfan syndrome
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Funding
- NCI NIH HHS [CA-34282, CA-78422] Funding Source: Medline
- NIAMS NIH HHS [AR-49698, AR-42044, AR-41135, AR-46811] Funding Source: Medline
- NIDCR NIH HHS [DE-13742] Funding Source: Medline
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Organismal physiology depends significantly on the proper assembly of extracellular matrix (ECM) macroaggregates that impart structural integrity to the connective tissue. Recent genetic studies in mice have unraveled unsuspected new functions of architectural matrix components in regulating signaling events that modulate patterning, morphogenesis, and growth of several organ systems. As a result, a new paradigm has emerged whereby tissue-specific organization of the ECM dictates not only the physical properties of the connective tissue, but also the ability of the matrix to direct a broad spectrum of cellular activities through the regulation of growth factor signaling. These observations pave the way to novel therapeutic approaches aimed at counteracting the deleterious consequences of perturbations of connective tissue homeostasis.
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