Results 41 to 50 of about 12,218 (212)

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

Journal 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, Sabi Bang, Gary Martin   +2 more
doaj   +1 more source

Biogenesis and function of fibrillin assemblies [PDF]

Cell and Tissue Research, 2009
Fibrillin-1 and fibrillin-2 are large cysteine-rich glycoproteins that serve two key physiological functions: as supporting structures that impart tissue integrity and as regulators of signaling events that instruct cell performance. The structural role of fibrillins is exerted through the temporal and hierarchical assembly of microfibrils and elastic ...
Francesco, Ramirez, Lynn Y, Sakai
openaire   +2 more sources

Assembly of Epithelial Cell Fibrillins [PDF]

Journal 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, Simon, Marcia, Sakai, Lynn Y., Dzamba, Bette J., Keene, Douglas R., Isogai, Zenzo, Charbonneau, Noe L.   +6 more
openaire   +2 more sources

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

Frontiers 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, Kaiqing Liu, Danyao Nie, Jing Zhang, Liyun Zhang, Xinhua Liu, Jiantao Wang   +6 more
doaj   +1 more source

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

FEBS 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., Raynaud, Sandy, Mérida Berlanga, Ángel, Concepción, Juan Carlos de la   +3 more
openaire   +5 more sources

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

Respiratory 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, Tetsuichiro Inai, Tomoki Kosho, Tomomi Yamaguchi, Yoshinori Kawabata, Yuko Inai, Shogo Imamura, Sakiko Sanada   +7 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

Conserved Function of Fibrillin5 in the Plastoquinone-9 Biosynthetic Pathway in Arabidopsis and Rice

Frontiers in Plant Science, 2017
Plastoquinone-9 (PQ-9) is essential for plant growth and development. Recently, we found that fibrillin5 (FBN5), a plastid lipid binding protein, is an essential structural component of the PQ-9 biosynthetic pathway in Arabidopsis.
Eun-Ha Kim, Dae-Woo Lee, Kyeong-Ryeol Lee, Su-Jin Jung, Jong-Seong Jeon, Hyun Uk Kim   +5 more
doaj   +1 more source

Biomechanical stress provides a second hit in the establishment of BMP/TGFβ-related vascular disorders

Cell Stress, 2020
Cardiovascular disorders are still the leading cause for mortality in the western world and challenge economies with steadily increasing healthcare costs.
Christian Hiepen, Jerome Jatzlau, Petra Knaus   +2 more
doaj   +1 more source

Therapy for Myhre Syndrome: Goals, Misconceptions, and Current Agents

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre Syndrome (MYHRS, MIM #139210) is a rare, multisystem connective tissue disorder caused by recurrent heterozygous gain‐of‐function pathogenic variants in the SMAD4 gene, a key player in TGF‐β signaling and a regulator of extracellular matrix homeostasis.
Alessandro De Falco, Alfonso Manuel D'Alessio, Nicola Brunetti‐Pierri   +2 more
wiley   +1 more source