Fibrillin: from microfibril assembly to biomechanical function

Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences
Cay M KieltyC Adrian Shuttleworth

Abstract

Fibrillins form the structural framework of a unique and essential class of extracellular microfibrils that endow dynamic connective tissues with long-range elasticity. Their biological importance is emphasized by the linkage of fibrillin mutations to Marfan syndrome and related connective tissue disorders, which are associated with severe cardiovascular, ocular and skeletal defects. These microfibrils have a complex ultrastructure and it has proved a major challenge both to define their structural organization and to relate it to their biological function. However, new approaches have at last begun to reveal important insights into their molecular assembly, structural organization and biomechanical properties. This paper describes the current understanding of the molecular assembly of fibrillin molecules, the alignment of fibrillin molecules within microfibrils and the unique elastomeric properties of microfibrils.

References

Jul 25, 1991·Nature·C L MaslenL Y Sakai
Jan 1, 1991·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·R FleischmajerT Faraggiana
Apr 1, 1991·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·D R KeeneR W Glanville
Sep 1, 1988·Journal of Ultrastructure and Molecular Structure Research·D W Wright, R Mayne
Dec 1, 1986·The Journal of Cell Biology·L Y SakaiE Engvall
Jan 1, 1983·International Review of Connective Tissue Research·E G Cleary, M A Gibson
Aug 1, 1995·The International Journal of Biochemistry & Cell Biology·C M Kielty, C A Shuttleworth
Dec 27, 1993·FEBS Letters·C M Kielty, C A Shuttleworth
Apr 26, 1996·Journal of Molecular Biology·D P ReinhardtL Y Sakai
Aug 9, 1996·The Journal of Biological Chemistry·D P ReinhardtL Y Sakai
Sep 15, 1996·Experientia·C J McConnellM E DeMont
Mar 14, 1997·The Journal of Biological Chemistry·D P ReinhardtH P Bächinger
Aug 1, 1997·The International Journal of Biochemistry & Cell Biology·M J SherrattC M Kielty
Jun 13, 1998·The Journal of Cell Biology·T J WessC M Kielty
Oct 29, 1998·Protein Science : a Publication of the Protein Society·X YuanK Downing
Jan 29, 1999·Matrix Biology : Journal of the International Society for Matrix Biology·S SinhaC M Kielty
Mar 20, 1999·The Journal of Biological Chemistry·T M RittyR P Mecham
Jan 14, 2000·Journal of Medical Genetics·P N Robinson, M Godfrey
Jan 26, 2000·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·S L DallasL F Bonewald
Aug 11, 2000·Micron : the International Research and Review Journal for Microscopy·M J SherrattC M Kielty
Oct 26, 2000·The British Journal of Ophthalmology·J L AshworthD McLeod
Nov 9, 2000·Matrix Biology : Journal of the International Society for Matrix Biology·P A HandfordL Y Sakai
Mar 10, 2001·The Journal of Cell Biology·C BaldockC M Kielty

❮ Previous
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Citations

Aug 30, 2011·Histochemistry and Cell Biology·Anthony J HayesJames Melrose
Jul 15, 2009·Cell and Tissue Research·Xiaojie XianJohn R Couchman
Apr 16, 2011·Journal of Mammary Gland Biology and Neoplasia·Anupama ChandramouliPamela Cowin
Jun 23, 2011·Journal of Cardiovascular Translational Research·Sonya B Seif-NaraghiKaren L Christman
Aug 30, 2003·Journal of Molecular Biology·Michael J SherrattCay M Kielty
Dec 14, 2002·Trends in Cell Biology·Benjamin S BrookeDean Y Li
Dec 23, 2003·The International Journal of Biochemistry & Cell Biology·Eichi TsurugaKazuharu Irie
Jun 15, 2005·Annual Review of Biochemistry·Luca JovinePaul M Wassarman
Nov 17, 2007·American Journal of Respiratory Cell and Molecular Biology·Stephen E McGowanTimothy M Ritty
Oct 25, 2007·Journal of Cardiothoracic Surgery·Rupert A PayneMartin A Denvir
Apr 2, 2011·American Journal of Veterinary Research·Steven G FriedenbergRory J Todhunter
Apr 2, 2014·Connective Tissue Research·Arabella Elizabeth Gardiner BairdAndrea D Short
Jul 17, 2013·The Journal of Surgical Research·Darrell WuScott A LeMaire
Oct 21, 2004·Birth Defects Research. Part C, Embryo Today : Reviews·Michael B FillaBrenda J Rongish
Apr 7, 2011·Journal of Anatomy·Kinley D SmithEithne J Comerford
Jan 10, 2012·Animal Genetics·T HiranoY Sugimoto
Mar 25, 2011·The Journal of Pathology·Boubacar MarikoGilles Faury
Dec 3, 2011·Chirality·Brigida Bochicchio, Antonietta Pepe
Feb 21, 2006·Comparative Biochemistry and Physiology. Part A, Molecular & Integrative Physiology·Chantal T BussiereM Edwin DeMont
Jul 16, 2015·International Journal of Experimental Pathology·John Gallagher
Jan 24, 2015·Experimental Eye Research·Samuel D HanlonAlan R Burns
Mar 4, 2008·Experimental Eye Research·Ted S Acott, Mary J Kelley
Jun 14, 2012·International Journal of Toxicology·Dal Mu Ri HanYoung Gyu Chai
Mar 27, 2007·Journal of Dental Research·E TsurugaT Yajima
Oct 8, 2016·Journal of Tissue Engineering and Regenerative Medicine·Karin V GrecoTahera Ansari
Apr 13, 2018·PloS One·Igor Yu DolmatovAlexey V Boyko
Dec 9, 2003·Laboratory Investigation; a Journal of Technical Methods and Pathology·Dionne LorenaAlexis Desmoulière
Mar 3, 2010·Laboratory Investigation; a Journal of Technical Methods and Pathology·Jürgen BrinckmannDieter P Reinhardt
Sep 8, 2011·Angewandte Chemie·Aaron M Kushner, Zhibin Guan
Jun 15, 2017·Journal of Biomedical Optics·George J TserevelakisGiannis Zacharakis
Dec 20, 2012·Circulation. Cardiovascular Genetics·Giuseppe MerlaMaria Nicla Loviglio
Dec 18, 2007·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Antoine A RobbesomToin H van Kuppevelt
Nov 6, 2014·Nature Reviews. Molecular Cell Biology·Janna K MouwValerie M Weaver
Nov 13, 2004·The Journal of Biological Chemistry·Severa BundaAleksander Hinek
Apr 18, 2020·Journal of Orthopaedic Research : Official Publication of the Orthopaedic Research Society·Marina DanalacheUlf K Hofmann
Oct 10, 2014·Journal of Biomolecular Screening·José PérezFrancisca Vicente
Apr 30, 2017·Biomedizinische Technik. Biomedical Engineering·Axel MalischewskiPetra Mela
Mar 25, 2004·The Journal of Biological Chemistry·Matthew J RockCay M Kielty

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