Amyloid β2-microglobulin is modified with Nε-(carboxymethyl)lysine in dialysis-related amyloidosis
نویسندگان
چکیده
منابع مشابه
Systemic Amyloidosis: Lessons from β2-Microglobulin*
β2-Microglobulin is responsible for systemic amyloidosis affecting patients undergoing long-term hemodialysis. Its genetic variant D76N causes a very rare form of familial systemic amyloidosis. These two types of amyloidoses differ significantly in terms of the tissue localization of deposits and for major pathological features. Considering how the amyloidogenesis of the β2-microglobulin mechan...
متن کاملIntermolecular Alignment in β2-Microglobulin Amyloid Fibrils
The deposition of amyloid-like fibrils, composed primarily of the 99-residue protein β2-microglobulin (β2m), is one of the characteristic symptoms of dialysis-related amyloidosis. Fibrils formed in vitro at low pH and low salt concentration share many properties with the disease related fibrils and have been extensively studied by a number of biochemical and biophysical methods. These fibrils c...
متن کاملCharacterization of the Response of Primary Cells Relevant to Dialysis-Related Amyloidosis to β2-Microglobulin Monomer and Fibrils
The formation of insoluble amyloid fibrils is associated with an array of devastating human diseases. Dialysis-related amyloidosis (DRA) is a severe complication of hemodialysis that results in the progressive destruction of the bones and joints. Elevated concentrations of β(2)-microglobulin (β(2)m) in the serum of subjects on hemodialysis promote the formation of amyloid fibrils in the osteoar...
متن کاملUnderstanding the complex mechanisms of β2-microglobulin amyloid assembly
Several protein misfolding diseases are associated with the conversion of native proteins into ordered protein aggregates known as amyloid. Studies of amyloid assemblies have indicated that non-native proteins are responsible for initiating aggregation in vitro and in vivo. Despite the importance of these species for understanding amyloid disease, the structural and dynamic features of amyloido...
متن کاملDialysis-related amyloidosis.
end-stage failure patients which has been recognized a Amyloid arthropathy decade ago. It generally occurs after several years of The deposition of b2M amyloid clearly is associated renal replacement therapy [1,2]. However, it may be with the syndrome of chronic arthralgias and arthropaseen occasionally in patients with long-standing, severe thy in dialysis patients, although the precise pathog...
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ژورنال
عنوان ژورنال: Kidney International
سال: 1996
ISSN: 0085-2538
DOI: 10.1038/ki.1996.442