New insights into the structural role of EMILINs within the human skin microenvironment
Supramolecular extracellular matrix (ECM) networks play an essential role in skin architecture and function. Elastin microfibril interface-located proteins (EMILINs) comprise a family of three extracellular glycoproteins that serve as essential ...
Alvise Schiavinato+15 more
doaj +1 more source
Microfibril-associated Glycoprotein-2 (MAGP-2) Is Specifically Associated with Fibrillin-containing Microfibrils but Exhibits More Restricted Patterns of Tissue Localization and Developmental Expression Than Its Structural Relative MAGP-1 [PDF]
Mark A. Gibson+3 more
openalex +1 more source
Structure of the Mutant Fibrillin-1 Gene in the Tight Skin (TSK) Mouse [PDF]
C Bona
openalex +1 more source
BackgroundMarfan syndrome (MFS) is a heritable disorder of connective tissue resulting from pathogenic variants of the fibrillin-1 gene (FBN1). Neonatal Marfan syndrome (nMFS) is rare and the most severe form of MFS, involving rapidly progressive ...
Qian Peng+3 more
semanticscholar +1 more source
P1148A in fibrillin-1 is not a mutation anymore [PDF]
Mei Wang+7 more
openaire +3 more sources
The Microfibrillar Proteins MAGP-1 and Fibrillin-1 Form a Ternary Complex with the Chondroitin Sulfate Proteoglycan Decorin [PDF]
Barbara Crippes Trask+3 more
openalex +1 more source
Mutations in Calcium-binding Epidermal Growth Factor Modules Render Fibrillin-1 Susceptible to Proteolysis [PDF]
Dieter P. Reinhardt+5 more
openalex +1 more source
Fibrillin-1 (FBN-1) a new marker of germ cell neoplasia in situ
BackgroundGerm cell neoplasia in situ (GCNIS), is preinvasive stage of testicular germ cell tumours (TGCTs). Fibrillins, which are integral components of microfibrils are suggested to be involved in cancer pathogenesis and maintenance of embryonic stem ...
Z. Cierna+11 more
semanticscholar +1 more source
The Biomechanics of Fibrillin Microfibrils: Lessons from the Ciliary Zonule
Marfan syndrome is an inherited connective tissue disorder that affects the cardiovascular, musculoskeletal, and ocular systems. It is caused by pathogenic variants in the fibrillin-1 gene (FBN1).
Pooja Rathaur+6 more
doaj +1 more source
Glycation is an ageing reaction of naturally occurring sugars with dermal proteins, with clinical signs appearing in vivo around age 30, and increasing steadily/regularly with age.
Seoungwoo Shin+5 more
doaj +1 more source