Cargando…

The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology

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. Mutations in fibrillin-1 cause pleiotropic manifestations in Marfan syndrome (MFS), in...

Descripción completa

Detalles Bibliográficos
Autores principales:	Asano, Keiichi, Cantalupo, Anna, Sedes, Lauriane, Ramirez, Francesco
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2022
Materias:	Review
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8836826/ https://www.ncbi.nlm.nih.gov/pubmed/35163812 http://dx.doi.org/10.3390/ijms23031892

Ejemplares similares

Pathophysiology and Therapeutics of Thoracic Aortic Aneurysm in Marfan Syndrome
por: Asano, Keiichi, et al.
Publicado: (2022)

The Supramolecular Organization of Fibrillin-Rich Microfibrils
por: Baldock, Clair, et al.
Publicado: (2001)

Fibrillin Microfibrils Keep the Cornea in Shape
por: Chakravarti, Shukti
Publicado: (2017)

Calcium Determines the Supramolecular Organization of Fibrillin-rich Microfibrils
por: Wess, T.J., et al.
Publicado: (1998)

Structural and compositional diversity of fibrillin microfibrils in human tissues
por: Eckersley, Alexander, et al.
Publicado: (2018)

Multiscale Imaging Reveals the Hierarchical Organization of Fibrillin Microfibrils
por: Godwin, Alan R.F., et al.
Publicado: (2018)

Fell‐Muir Lecture: Fibrillin microfibrils: structural tensometers of elastic tissues?
por: Kielty, Cay M.
Publicado: (2017)

Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development
por: Mead, Timothy J, et al.
Publicado: (2022)

Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils
Publicado: (1995)

New Insights into the Assembly of Extracellular Microfibrils from the Analysis of the Fibrillin 1 Mutation in the Tight skin Mouse
por: Gayraud, Barbara, et al.
Publicado: (2000)

Extracellular Regulation of Bone Morphogenetic Protein Activity by the Microfibril Component Fibrillin-1
por: Wohl, Alexander P., et al.
Publicado: (2016)

Assessment of Bones Deficient in Fibrillin-1 Microfibrils Reveals Pronounced Sex Differences
por: Altinbas, Lukas, et al.
Publicado: (2019)

Epithelial–mesenchymal status influences how cells deposit fibrillin microfibrils
por: Baldwin, Andrew K., et al.
Publicado: (2014)

The role of fibrillin and microfibril binding proteins in elastin and elastic fibre assembly
por: Godwin, Alan R.F., et al.
Publicado: (2019)

Fibrillin microfibrils and elastic fibre proteins: Functional interactions and extracellular regulation of growth factors
por: Thomson, Jennifer, et al.
Publicado: (2019)

The Tight Skin Mouse: Demonstration of Mutant Fibrillin-1 Production and Assembly into Abnormal Microfibrils
por: Kielty, Cay M., et al.
Publicado: (1998)

Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils
Publicado: (1986)

Altered Ocular Fibrillin Microfibril Composition in Mice With a Glaucoma-Causing Mutation of Adamts10
por: Wu, Hang-Jing, et al.
Publicado: (2021)

Dissecting aortic aneurysm in Marfan syndrome is associated with losartan-sensitive transcriptomic modulation of aortic cells
por: Sun, Yifei, et al.
Publicado: (2023)

Structure of the Fibrillin-1 N-Terminal Domains Suggests that Heparan Sulfate Regulates the Early Stages of Microfibril Assembly
por: Yadin, David A., et al.
Publicado: (2013)

Gremlin-1 associates with fibrillin microfibrils in vivo and regulates mesothelioma cell survival through transcription factor slug
por: Tamminen, J A, et al.
Publicado: (2013)

Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta
por: Șulea, Cristina M., et al.
Publicado: (2023)

Assembly assay identifies a critical region of human fibrillin-1 required for 10–12 nm diameter microfibril biogenesis
por: Jensen, Sacha A., et al.
Publicado: (2021)

Fibrillin assemblies: extracellular determinants of tissue formation and fibrosis
por: Olivieri, Jacopo, et al.
Publicado: (2010)

Using measures of single‐cell physiology and physiological state to understand organismic aging
por: Mendenhall, Alexander, et al.
Publicado: (2015)

Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia – a novel mouse model providing insights into geleophysic dysplasia
por: Hubmacher, Dirk, et al.
Publicado: (2015)

Fibrillin microfibril structure identifies long-range effects of inherited pathogenic mutations affecting a key regulatory latent TGFβ-binding site
por: Godwin, Alan R. F., et al.
Publicado: (2023)

Fibrillins in Tendon
por: Giusti, Betti, et al.
Publicado: (2016)

Regulation of limb patterning by extracellular microfibrils
por: Arteaga-Solis, Emilio, et al.
Publicado: (2001)

Microenvironmental Regulation by Fibrillin-1
por: Sengle, Gerhard, et al.
Publicado: (2012)

POGLUT2 and POGLUT3 O-glucosylate multiple EGF repeats in fibrillin-1, -2, and LTBP1 and promote secretion of fibrillin-1
por: Williamson, Daniel B., et al.
Publicado: (2021)

The Evolution of Extracellular Fibrillins and Their Functional Domains
por: Piha-Gossack, Adam, et al.
Publicado: (2012)

Publisher Correction: Fibrillin microfibril structure identifies long-range effects of inherited pathogenic mutations affecting a key regulatory latent TGFβ-binding site
por: Godwin, Alan R. F., et al.
Publicado: (2023)

LncRNA Functional Screening in Organismal Development
por: Li, Yang, et al.
Publicado: (2023)

Organismal Design and Biomimetics: A Problem of Scale
por: Perricone, Valentina, et al.
Publicado: (2021)

THE SIZE OF THE CELLULOSE MICROFIBRIL
por: Colvin, J. Ross
Publicado: (1963)

ON THE DIMENSIONS OF CELLULOSE MICROFIBRILS
por: Ohad, I., et al.
Publicado: (1964)

Organismal Roles of Hsp90
por: van Oosten-Hawle, Patricija
Publicado: (2023)

The Effect of Ultraviolet B on Fibrillin-1 and Fibrillin-2 in Human Non-pigmented Ciliary Epithelial Cells In Vitro
por: Shiroto, Yuki, et al.
Publicado: (2017)

Fibrillin-1 Regulates Arteriole Integrity in the Retina
por: Alonso, Florian, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_st6k9iise2rsg9ikbvi0fttj12