Results 21 to 30 of about 9,961 (164)

Fibrillines et fibrillinopathies [PDF]

open access: yesmédecine/sciences, 1996
Microfibrils contain a variety of proteins, the most prominent of which are the two fibrillins. Fibrillins are large glycoproteins (320 kDa) ubiquitously distributed in connective tissues. Together with amorphous elastin, fibrillin-containing microfibrils form the elastic fibers.
Collod-Beroud, Gwenaëlle   +1 more
openaire   +2 more sources

"Computational Analysis of the Effect of fbn1 Gene Mutations in the Marfan Syndrome" [PDF]

open access: yesIranian Journal of Public Health, 2004
Fibrillin is a large glycoprotein synthesized in the tissues involved in Marfan syndrome, and known to be involved in tissue elasticity. The syndrome is corresponded to fbn1 gene and is characterized by cardiovascular, ocular, and skeletal abnormalities.
H Mohabatkar
doaj   +2 more sources

Assembly of Epithelial Cell Fibrillins [PDF]

open access: yesJournal of Investigative Dermatology, 2001
Fibrillins are large structural macromolecules that are components of connective tissue microfibrils. Fibrillin microfibrils have been found in association with basement membranes, where microfibrils appear to insert directly into the lamina densa.
Karaman-Jurukovska, Nevena   +6 more
openaire   +2 more sources

The ADAMTS/Fibrillin Connection: Insights into the Biological Functions of ADAMTS10 and ADAMTS17 and Their Respective Sister Proteases

open access: yesBiomolecules, 2020
Secreted a disintegrin-like and metalloprotease with thrombospondin type 1 motif (ADAMTS) proteases play crucial roles in tissue development and homeostasis.
Stylianos Z. Karoulias   +3 more
doaj   +1 more source

Arabidopsis thaliana plastoglobule‐associated fibrillin 1a interacts with fibrillin 1b in vivo [PDF]

open access: yesFEBS Letters, 2014
Plant fibrillins are a well‐conserved protein family found in the plastids of all photosynthetic organisms, where they perform a wide range of functions. A number of these proteins have been suggested to be involved in the maintenance of thylakoids and the formation of plastoglobules, preventing their coalescence and favoring their clustering via an as‐
Gámez Arjona, Francisco M.   +3 more
openaire   +5 more sources

When proteomics reveals unsuspected roles: the plastoglobule example

open access: yesFrontiers in Plant Science, 2013
Plastoglobules are globular compartments found in plastids. Before initial proteomic studies were published, these particles were often viewed as passive lipid droplets whose unique role was to store lipids coming from the thylakoid turn-over, or to ...
Claire eBréhélin, Houda eNacir
doaj   +1 more source

Microscopic Anatomy of the Lining of Hemal Spaces in the Penaeid Shrimp, Sicyonia ingentis

open access: yesJournal of Marine Science and Engineering, 2021
The purpose of this paper is to present a morphological description of three different types of acellular material lining hemal spaces in a shrimp, providing a background for addressing future questions.
Rachel Brittany Sidebottom   +2 more
doaj   +1 more source

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

open access: yesJournal 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

Case report: Biochemical and clinical phenotypes caused by cysteine substitutions in the epidermal growth factor-like domains of fibrillin-1

open access: yesFrontiers in Genetics, 2022
Marfan syndrome, an autosomal dominant disorder of connective tissue, is primarily caused by mutations in the fibrillin-1 (FBN1) gene, which encodes the protein fibrillin-1.
Xin Liu   +6 more
doaj   +1 more source

A patient with pleuroparenchymal fibroelastosis carrying a novel fibrillin-2 gene variant

open access: yesRespiratory Medicine Case Reports, 2023
Pleuroparenchymal fibroelastosis is a recently recognized clinical entity characterized by interstitial pneumonia with proliferating elastin in the upper lung regions. Pleuroparenchymal fibroelastosis is categorized as idiopathic or reported depending on
Kouko Hidaka   +7 more
doaj   +1 more source

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