Extracellular microfibrils in development and disease

F. Ramirez, Lynn Sakai, D. B. Rifkin, H. C. Dietz

Research output: Contribution to journalReview article

37 Citations (Scopus)

Abstract

Fibrillins are the structural components of extracellular microfibrils that impart physical properties to tissues, alone or together with elastin as elastic fibers. Genetic studies in mice have revealed that fibrillin-rich microfibrils are also involved in regulating developmental programs and homeostatic processes through the modulation of TGF-β/BMP signaling events. A new paradigm has thus emerged whereby the spatiotemporal organization of microfibrils dictates both the cellular activities and physical properties of connective tissues. These observations have paved the way to novel therapeutic approaches aimed at counteracting the life-threatening complications in human conditions caused by dysfunctions of fibrillin-rich microfibrils.

Original languageEnglish (US)
Pages (from-to)2437-2446
Number of pages10
JournalCellular and Molecular Life Sciences
Volume64
Issue number18
DOIs
StatePublished - Sep 1 2007
Externally publishedYes

Fingerprint

Microfibrils
Elastic Tissue
Elastin
Connective Tissue
Fibrillins

Keywords

  • Aortic aneurysm
  • Development and morphogenesis
  • Emphysema
  • Extracellular matrix
  • Fibrillin
  • Marfan syndrome
  • Myopathy
  • TGF-β/BMP signaling molecules

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology
  • Pharmacology
  • Cellular and Molecular Neuroscience
  • Cell Biology

Cite this

Extracellular microfibrils in development and disease. / Ramirez, F.; Sakai, Lynn; Rifkin, D. B.; Dietz, H. C.

In: Cellular and Molecular Life Sciences, Vol. 64, No. 18, 01.09.2007, p. 2437-2446.

Research output: Contribution to journalReview article

Ramirez, F. ; Sakai, Lynn ; Rifkin, D. B. ; Dietz, H. C. / Extracellular microfibrils in development and disease. In: Cellular and Molecular Life Sciences. 2007 ; Vol. 64, No. 18. pp. 2437-2446.
@article{6ce133474bd84afd8682ad3b87633200,
title = "Extracellular microfibrils in development and disease",
abstract = "Fibrillins are the structural components of extracellular microfibrils that impart physical properties to tissues, alone or together with elastin as elastic fibers. Genetic studies in mice have revealed that fibrillin-rich microfibrils are also involved in regulating developmental programs and homeostatic processes through the modulation of TGF-β/BMP signaling events. A new paradigm has thus emerged whereby the spatiotemporal organization of microfibrils dictates both the cellular activities and physical properties of connective tissues. These observations have paved the way to novel therapeutic approaches aimed at counteracting the life-threatening complications in human conditions caused by dysfunctions of fibrillin-rich microfibrils.",
keywords = "Aortic aneurysm, Development and morphogenesis, Emphysema, Extracellular matrix, Fibrillin, Marfan syndrome, Myopathy, TGF-β/BMP signaling molecules",
author = "F. Ramirez and Lynn Sakai and Rifkin, {D. B.} and Dietz, {H. C.}",
year = "2007",
month = "9",
day = "1",
doi = "10.1007/s00018-007-7166-z",
language = "English (US)",
volume = "64",
pages = "2437--2446",
journal = "Cellular and Molecular Life Sciences",
issn = "1420-682X",
publisher = "Birkhauser Verlag Basel",
number = "18",

}

TY - JOUR

T1 - Extracellular microfibrils in development and disease

AU - Ramirez, F.

AU - Sakai, Lynn

AU - Rifkin, D. B.

AU - Dietz, H. C.

PY - 2007/9/1

Y1 - 2007/9/1

N2 - Fibrillins are the structural components of extracellular microfibrils that impart physical properties to tissues, alone or together with elastin as elastic fibers. Genetic studies in mice have revealed that fibrillin-rich microfibrils are also involved in regulating developmental programs and homeostatic processes through the modulation of TGF-β/BMP signaling events. A new paradigm has thus emerged whereby the spatiotemporal organization of microfibrils dictates both the cellular activities and physical properties of connective tissues. These observations have paved the way to novel therapeutic approaches aimed at counteracting the life-threatening complications in human conditions caused by dysfunctions of fibrillin-rich microfibrils.

AB - Fibrillins are the structural components of extracellular microfibrils that impart physical properties to tissues, alone or together with elastin as elastic fibers. Genetic studies in mice have revealed that fibrillin-rich microfibrils are also involved in regulating developmental programs and homeostatic processes through the modulation of TGF-β/BMP signaling events. A new paradigm has thus emerged whereby the spatiotemporal organization of microfibrils dictates both the cellular activities and physical properties of connective tissues. These observations have paved the way to novel therapeutic approaches aimed at counteracting the life-threatening complications in human conditions caused by dysfunctions of fibrillin-rich microfibrils.

KW - Aortic aneurysm

KW - Development and morphogenesis

KW - Emphysema

KW - Extracellular matrix

KW - Fibrillin

KW - Marfan syndrome

KW - Myopathy

KW - TGF-β/BMP signaling molecules

UR - http://www.scopus.com/inward/record.url?scp=34548632168&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=34548632168&partnerID=8YFLogxK

U2 - 10.1007/s00018-007-7166-z

DO - 10.1007/s00018-007-7166-z

M3 - Review article

VL - 64

SP - 2437

EP - 2446

JO - Cellular and Molecular Life Sciences

JF - Cellular and Molecular Life Sciences

SN - 1420-682X

IS - 18

ER -