Results 91 to 100 of about 19,229 (251)

Impact of N‐linked glycans on the dual short fibulin/LTBP‐4 axes regulating elastogenesis

The FEBS Journal, EarlyView.
Elastic fiber assembly is orchestrated by multiple glycoproteins. We delineate the molecular basis of two key axes involved in elastic fiber formation—LTBP‐4L/fibulin‐4 and LTBP‐4S/fibulin‐5. We show that N‐linked glycans on these glycoproteins regulate their interactions and conformations, both of which are critical aspects in elastic fiber formation.
Valentin Nelea, Heena Kumra, Chae Syng Lee, Daniel Williamson, Hana Hakami, Rong‐Mo Zhang, Neha E. H. Dinesh, Robert Haltiwanger, Dieter P. Reinhardt   +8 more
wiley   +1 more source

Fibrillin–integrin interactions in health and disease

Biochemical Society Transactions, 2008
Human fibrillin-1 is the major structural protein of extracellular matrix 10–12 nm microfibrils. It has a disulfide-rich modular organization which consists primarily of cbEGF (Ca2+-binding epidermal growth factor-like) domains and TB (transforming growth factor β-binding protein-like) domains.
Jovanovic, J, Iqbal, S, Jensen, S, Mardonf, H, Handford, P   +4 more
openaire   +5 more sources

The structural role of elastic fibres in the cornea investigated using a mouse model for Marfan syndrome [PDF]

, 2017
Purpose: The presence of fibrillin-rich elastic fibers in the cornea has been overlooked in recent years. The aim of the current study was to elucidate their functional role using a mouse model for Marfan syndrome, defective in fibrillin-1, the major ...
Bell, James Stephen, Farinha, L., Fergusson, James R., Hayes, Sally, Lewis, Philip, Meek, Keith Michael Andrew, Pereira, L. V., White, Nicholas, White, Tomas   +8 more
core   +1 more source

Higher blood pressure in elderly hypertensive females, with increased arterial stiffness and blood pressure in females with the Fibrillin-1 2/3 genotype

BMC Cardiovascular Disorders, 2020
Background Elderly patients have a relatively high cardiovascular risk due to increased arterial stiffness, elevated blood pressure and decreased amounts of elastin in the arteries.
Ida Åström Malm, Urban Alehagen, Peter Blomstrand, Ulf Dahlström, Rachel De Basso   +4 more
doaj   +1 more source

Characterisation of spinal ligaments in the embryonic chick

Journal of Anatomy, EarlyView.
Use of the embryonic chick provides an opportunity to examine the maturation of spinal ligaments during development, to inform anatomical defects in the spine. Abstract Ligaments are important connective tissues within the musculoskeletal system that connect bone to bone and provide support and stability.
Sarah Hennigan, Ebru Talak Basturkmen, Rebecca A. Rolfe   +2 more
wiley   +1 more source

The role of calcium in the organization of fibrillin microfibrils

FEBS Letters, 1993
The microfibrillar glycoprotein fibrillin has a multidomain structure which contains forty‐three epidermal growth factor‐like motifs with calciumbinding consensus sequences. We have utilized intact microfibrils isolated from human dermal fibroblast cultures to investigate the putative influence of bound calcium on microfibrillar organization and ...
Kielty, CM, Shuttleworth, CA
openaire   +4 more sources

Antifibrotic effects of specific targeting of the 5‐hydroxytryptamine 2B receptor (5‐HT2BR) in murine models and ex vivo models of scleroderma skin

Arthritis &Rheumatology, Volume 77, Issue 8, Page 1063-1076, August 2025.
Objective Systemic sclerosis (SSc) is a connective tissue disease with fibrotic remodeling of the skin and various internal organs. SSc is associated with the highest case‐specific mortality of all rheumatic autoimmune diseases with limited antifibrotic treatment options.
Thuong Trinh‐Minh, Cuong Tran‐Manh, Andrea‐Hermina Györfi, Nicholas Dickel, Christoph Liebel, Xiang Zhou, Jiucun Wang, Meik Kunz, Helena Arozenius, Lars Pettersson, Sam Lindgren, Christina Wenglén, Jörg H. W. Distler   +12 more
wiley   +1 more source

Comprehensive clinical and molecular analysis of 12 families with type 1 recessive cutis laxa. [PDF]

, 2013
Autosomal recessive cutis laxa type I (ARCL type I) is characterized by generalized cutis laxa with pulmonary emphysema and/or vascular complications. Rarely, mutations can be identified in FBLN4 or FBLN5.
Bresson, J.L., Callewaert, B., Coucke, P.J., Davis, E.C., De Almeida, S., De Paepe, A., Gaspar, H., Gosendi, M.E., Heimdal, K., Kornak, U., Lee, J.G., Mac Neal, M., Madan-Khetarpal, S., Malfait, F., Mansour, S., Piérard, G.E., Prescott, K., Salhi, A., Schulz, B., Sciurba, F.C., Su, C.T., Symoens, S., Unger, S., Urban, Z., Van Damme, T., Van Maldergem, L., Vanakker, O., Vlummens, P.   +27 more
core   +1 more source

Genome-Wide Identification and Expression Analyses of the Fibrillin Family Genes Suggest Their Involvement in Photoprotection in Cucumber

Plants, 2018
Fibrillin (FBN) is a plastid lipid-associated protein found in photosynthetic organisms from cyanobacteria to plants. In this study, 10 CsaFBN genes were identified in genomic DNA sequences of cucumber (Chinese long and Gy14) through database searches ...
Inyoung Kim, Sang-Choon Lee, Eun-Ha Kim, Kihwan Song, Tae-Jin Yang, Hyun Uk Kim   +5 more
doaj   +1 more source

Cooperative Mechanism of ADAMTS/ ADAMTSL and Fibrillin-1 in the Marfan Syndrome and Acromelic Dysplasias

Frontiers in Genetics, 2021
The term “fibrillinopathies” gathers various diseases with a wide spectrum of clinical features and severity but all share mutations in the fibrillin genes.
Pauline Arnaud, Pauline Arnaud, Zakaria Mougin, Catherine Boileau, Catherine Boileau, Carine Le Goff   +5 more
doaj   +1 more source