Results 71 to 80 of about 23,678,950 (225)
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+12 more
wiley +1 more source
Calcium Stabilizes Fibrillin-1 against Proteolytic Degradation [PDF]
The calcium-binding epidermal growth factor (cbEGF)-like domain is a structural motif that is present in many matrix proteins throughout the animal kingdom from invertebrates to mammals. This module has been demonstrated to bind calcium in the micromolar range.
Robert N. Ono+3 more
openaire +3 more sources
A novel mutation in FBN1 gene in autosomal dominant Marfan syndrome and macular degeneration in a Chinese consanguineous family [PDF]
AIM: To report a novel mutation in FBN1 gene in a Chinese consanguineous family with common Marfan syndrome (MFS) phenotype and an unusual bilateral macular degeneration.
Ping-Bo Ouyang+5 more
doaj +1 more source
Use of Liquid Nitrogen in Food Products: A Review
ABSTRACT The increasing demand for frozen products has led companies to offer a variety of foods that may struggle to maintain quality. Traditional freezing methods often negatively impact food quality, affecting texture, color, flavor, and so forth.
Fabiola Pesce+3 more
wiley +1 more source
Frequent bone fractures in infancy require the elimination of a large number (> 100) of genetic disorders. The modern diagnostic method of hereditary diseases characterized by debilitating course is a new generation sequencing.
G. T. Yakhyaeva+5 more
doaj +1 more source
The figure presents the trilaminar organization of the human deep fascia, composed of a thick, collagen‐rich intermediate layer flanked by thinner superficial and deep layers with loose connective tissue. ABSTRACT Traditional histological methods provide limited insights into the complex 3D microstructure of fascia and its relationship to disease. This
Chiedozie Kenneth Ugwoke+6 more
wiley +1 more source
ADAMTSL6β Protein Rescues Fibrillin-1 Microfibril Disorder in a Marfan Syndrome Mouse Model through the Promotion of Fibrillin-1 Assembly [PDF]
Marfan syndrome (MFS) is a systemic disorder of the connective tissues caused by insufficient fibrillin-1 microfibril formation and can cause cardiac complications, emphysema, ocular lens dislocation, and severe periodontal disease. ADAMTSL6β (A disintegrin-like metalloprotease domain with thrombospondin type I motifs-like 6β) is a microfibril ...
Kazutaka Kosaka+17 more
openaire +4 more sources
Stress in dipteran insects mass‐reared for sterile insect technique applications
The connections between biotic and abiotic stress affecting mass‐reared dipteran insects and the associated stress and immunological responses. Numbers indicate the order in which the topics are discussed in this review. Abstract Stress may be viewed as the disturbance of homeostasis of an organism.
Caroline K. Mirieri+3 more
wiley +1 more source
The organisation of elastin and fibrillins 1 and 2 in the cruciate ligament complex [PDF]
Although elastin fibres and oxytalan fibres (bundles of microfibrils) have important mechanical, biochemical and cell regulatory functions, neither their distribution nor their function in cruciate ligaments has been investigated. Twelve pairs of cruciate ligaments (CLs) were obtained from 10 adult dogs with no evidence of knee osteoarthritis.
Smith, Kinley D.+5 more
openaire +4 more sources
The Diversity of Fibrillin Functions: Lessons from the Periodontal Ligament
Marfan syndrome is caused by a mutation in the FBN1 gene encoding fibrillin-1. This extracellular matrix glycoprotein, which assembles into microfibrils, is best known for its scaffolding role in the production of elastic fibers responsible for ...
Elisabeth Genot+4 more
doaj +1 more source