Results 21 to 30 of about 21,236,271 (159)

Fibulin-5 interacts with fibrillin-1 molecules and microfibrils. [PDF]

Biochemical Journal, 2005
Fibulin-5 plays an important role in elastic fibre formation in vivo. We have investigated the molecular interactions between fibulin-5 and components of fibrillin-rich microfibrils which form a template for elastin.
Hodson, Nigel, Sherratt, MJ, Freeman, LJ, Kielty, Cay M., Shuttleworth, Adrian, Lomas, A, Weiss, AS, Shuttleworth, A, Lomas, Amanda, Kielty, CM, Sherratt, Michael J.; id_orcid, Hodson, N, Freeman, Lyle J., Mellody, KT, Mellody, Kieran T., Weiss, Anthony S.   +15 more
core   +4 more sources

ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly [PDF]

Journal of Biological Chemistry, 2011
This research was originally published in the Journal of Biological Chemistry. Masahiro Saito, Misaki Kurokawa, Masahito Oda, Masamitsu Oshima, Ko Tsutsui, Kazutaka Kosaka, Kazuhisa Nakao, Miho Ogawa, Ri-ichiroh Manabe, Naoto Suda, Ganburged Ganjargal ...
Ganjargal, Ganburged, Manabe, Ri-ichiroh, Oshima, Masamitsu, Tsutsui, Ko, Tsuji, Takashi, Nakao, Kazuhisa, Sekiguchi, Kiyotoshi, Teranaka, Toshio, Kurokawa, Misaki, Noguchi, Toshihide, Suda, Naoto, Hada, Yasunobu, Saito, Masahiro, Kosaka, Kazutaka, Ogawa, Miho, Yoneda, Toshiyuki, Oda, Masahito   +16 more
core   +5 more sources

The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology [PDF]

International Journal of Molecular Sciences, 2022
Fibrillin-1 is the major structural component of the 10 nm-diameter microfibrils that confer key physical and mechanical properties to virtually every tissue, alone and together with elastin in the elastic fibers.
Keiichi Asano, Lauriane Sedes, Francesco Ramirez, Anna Cantalupo   +3 more
core   +3 more sources

Fibrillin-1, a calcium binding protein of extracellular matrix.

Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2000
Fibrillin-1 is a large extracellular matrix glycoprotein which assembles to form 10-12 nm microfibrils in extracellular matrix. Mutations in the human fibrillin-1 gene (FBN-1) cause the connective tissue disease Marfan syndrome and related disorders ...
P.A Handford, Handford, Penelope, Handford, P.A, Handford, PA   +3 more
core   +3 more sources

Aqueous humor TGFβ and fibrillin-1 in Tsk mice reveal clues to POAG pathogenesis

Scientific Reports
Aqueous humor (AH) and blood levels of transforming growth factor β (TGFβ) are elevated in idiopathic primary open angle glaucoma (POAG) representing a disease biomarker of unclear status and function.
James C. Tan, MinHee K. Ko, Jeong-Im Woo, Kenneth L. Lu, Jonathan A. Kelber   +4 more
doaj   +2 more sources

The extracellular matrix glycoprotein fibrillin-1 in health and disease

Frontiers in Cell and Developmental Biology
Fibrillin-1 (FBN1) is a large, cysteine-rich, calcium binding extracellular matrix glycoprotein encoded by FBN1 gene. It serves as a structural component of microfibrils and provides force-bearing mechanical support in elastic and nonelastic connective ...
Li Li, Li Li, Junxin Huang, Junxin Huang, Youhua Liu, Youhua Liu   +5 more
doaj   +3 more sources

Macromolecular crowding enhances fibrillin-1 deposition in the extracellular matrix [PDF]

European Cells & Materials, 2022
Biochemical and biophysical factors need consideration when modelling in vivo cellular behaviour using in vitro cell culture systems. One underappreciated factor is the high concentration of macromolecules present in vivo, which is typically not ...
B Satz-Jacobowitz, N Taye, SZ Karoulias, D Hubmacher   +3 more
doaj   +1 more source

Fibrillin-1 and fibrillin-1-derived asprosin in adipose tissue function and metabolic disorders [PDF]

Journal of Cell Communication and Signaling, 2020
The extracellular matrix microenvironment of adipose tissue is of critical importance for the differentiation, remodeling and function of adipocytes. Fibrillin-1 is one of the main components of microfibrils and a key player in this process. Furin processing of profibrillin-1 results in mature fibrillin-1 and releases the C-terminal propeptide as a ...
Muthu L. Muthu, Dieter P. Reinhardt
openaire   +2 more sources

Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development

eLife, 2022
The embryonic extracellular matrix (ECM) undergoes transition to mature ECM as development progresses, yet few mechanisms ensuring ECM proteostasis during this period are known.
Timothy J Mead, Daniel R Martin, Lauren W Wang, Stuart A Cain, Cagri Gulec, Elisabeth Cahill, Joseph Mauch, Dieter Reinhardt, Cecilia Lo, Clair Baldock, Suneel S Apte   +10 more
doaj   +1 more source

Circulating fibrillin fragment concentrations in patients with and without aortic pathology

JVS - Vascular Science, 2022
Objective: Fragments of fibrillin-1 and fibrillin-2 will be detectable in the plasma of patients with aortic dissections and aneurysms. We sought to determine whether the plasma fibrillin fragment levels (PFFLs) differ between patients with thoracic ...
Eric J. Carlson, PhD, Megan Rushkin, BA, Derek Darby, BA, Trisha Chau, BS, Renee L. Shirley, PhD, Jeff S. King, PhD, Khanh Nguyen, MD, Gregory J. Landry, MD, Gregory L. Moneta, MD, Cherrie Abraham, MD, Lynn Y. Sakai, PhD, Amir F. Azarbal, MD   +11 more
doaj   +1 more source