Fibrillin-1 Regulates Arteriole Integrity in the Retina [PDF]
Biomolecules, 2022 Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils that provide critical functions in large blood vessels and other tissues.
Florian Alonso +4 more
doaj +2 more sources
Case report: Biochemical and clinical phenotypes caused by cysteine substitutions in the epidermal growth factor-like domains of fibrillin-1 [PDF]
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 +6 more
doaj +2 more sources
The extracellular matrix glycoprotein fibrillin-1 in health and disease [PDF]
Frontiers in Cell and Developmental Biology, 2023 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 +5 more
doaj +2 more sources
Comparison of Fibrillin-1 and Fibrillin-2 Gene Expression Level in Esophageal Squamous Cell Carcinoma Tumor Tissue and Tumor Margin Tissue [PDF]
Iranian Journal of Medical SciencesBackground: Esophageal cancer is one of the most devastating cancers of the gastrointestinal tract. We will compare the expression levels of fibrillin-1 (FBN1) and fibrillin-2 (FBN2), a family of extracellular matrix glycoproteins, in esophageal cancer ...
Mahsa Mahdizadeh +3 more
doaj +2 more sources
Cooperative Mechanism of ADAMTS/ ADAMTSL and Fibrillin-1 in the Marfan Syndrome and Acromelic Dysplasias [PDF]
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 +5 more
doaj +2 more sources
Aqueous humor TGFβ and fibrillin-1 in Tsk mice reveal clues to POAG pathogenesis [PDF]
Scientific ReportsAqueous 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 +4 more
doaj +2 more sources
Fibrillin-1 regulates periostin expression during maintenance of periodontal homeostasis [PDF]
Journal of Dental Sciences, 2022 Background/purpose: Human periodontal ligament consists of elastic system fibers, mainly fibrillin-1 (FBN1). Periostin (POSTN) maintains periodontal homeostasis.
Yoshikazu Manabe +7 more
doaj +2 more sources
Sex specific knee joint soft tissue mineralization with fibrillin-1 mutation in male tight skin mice
European Cells & MaterialsBackground: Articular soft tissue mineralization and ossification are clear pathological signs of osteoarthritis (OA) joints. However their molecular and cellular aetiologies remain largely unknown.
C Keenan +8 more
doaj +2 more sources
Fibrillin-1 regulates endothelial sprouting during angiogenesis [PDF]
Proceedings of the National Academy of Sciences of the United States of America, 2023 Dieter Peter Reinhardt +2 more
exaly +2 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 +3 more
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