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Neuroferritinopathy is a rare, adult-onset, dominantly inherited movement disorder caused by mutations in ferritin gene. A ferritin light chain variant related to neuroferritinopathy, in which alanine 96 is replaced with threonine (A96T), was expressed in Escherichia coli, purified, and characterized. The circular dichroism spectrum, analytical ultracentrifuge, and small-angle X-ray scattering studies have shown that both subunit structure and assembly of A96T are the same as those of wild-type human ferritin light chain (HuFTL). Iron-incorporation ability was also comparable to that of HuFTL. Although the structural stability against heat, acid, and denaturant was reduced, the structure was sufficiently stable under physiological conditions. The most remarkable defect observed for A96T was lower refolding efficiency and higher propensity to aggregate. The possible relationship between folding deficiency and disease is discussed.
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Plant ferritin in holo form is considered as a novel, ideal iron supplement for human nutrition in the 21st century, but its self-degradation and self-association features limit its application on acc...
G protein-coupled receptors (GPCRs) contain highly hydrophobic domains that are subject to aggregation when exposed to the crowded environment of the cytoplasm. Many events can lead to protein aggrega...
Experimental determination of the key features of the free energy landscapes of proteins which dictate their adeptness to fold correctly, or propensity to misfold and aggregate, and which are modulate...
Ferritin is not only a biomarker of total iron status and systemic inflammation but is also associated with metabolic disorders. A number of genetic variations have been identified to affect serum fer...
Ferritin is the major iron storage molecule of vertebrates, which can be detected in serum under numerous conditions, including inflammatory, neurodegenerative, and malignant diseases. Given this char...
A serum ferritin level can reflect the total body iron content, thus a very low serum ferritin is commonly used as an indicator of iron deficiency and a very high serum ferritin is commonl...
This study investigates a new technology to assess the structure and function inside the eye. Retinal imaging of subjects with inner and outer retinal defects to detect areas of abnormal s...
Obesity is associated with alterations in brain structure and cognitive impairment and is a risk factor for Alzheimer's disease and vascular dementia. The mechanisms underlying obesity rel...
This study was designed to assess the relation between serum ferritin and hepatic fibrosis in patients with NAFLD. It included 83 patients with NAFLD with or without hepatic fibrosis, in a...
Myocardial iron overload is the leading cause of death in patients with beta-thalassemia major (TM). Therapy with deferoxamine (DFO) combined with deferiprone (DFP) reduces myocardial iron...
Stress-inducible members of the heat-shock proteins 70 family. HSP72 heat shock proteins function with other MOLECULAR CHAPERONES to mediate PROTEIN FOLDING and to stabilize pre-existent proteins against aggregation.
Body of knowledge related to the use of organisms, cells or cell-derived constituents for the purpose of developing products which are technically, scientifically and clinically useful. Alteration of biologic function at the molecular level (i.e., GENETIC ENGINEERING) is a central focus; laboratory methods used include TRANSFECTION and CLONING technologies, sequence and structure analysis algorithms, computer databases, and gene and protein structure function analysis and prediction.
A rapid biochemical reaction involved in the formation of proteins. It begins even before a protein has been completely synthesized and proceeds through discrete intermediates (primary, secondary, and tertiary structures) before the final structure (quaternary structure) is developed.
The processes of RNA tertiary structure formation.
Resemblance in appearance, structure, function, sound, scent or behavior between related or unrelated species, occurring in the same geographic location.
Bioinformatics is the application of computer software and hardware to the management of biological data to create useful information. Computers are used to gather, store, analyze and integrate biological and genetic information which can then be applied...
Radiology is the branch of medicine that studies imaging of the body; X-ray (basic, angiography, barium swallows), ultrasound, MRI, CT and PET. These imaging techniques can be used to diagnose, but also to treat a range of conditions, by allowing visuali...