Advanced glycation end product in familial amyloidotic polyneuropathy (FAP)
نویسندگان
چکیده
منابع مشابه
Argpyrimidine, a methylglyoxal-derived advanced glycation end-product in familial amyloidotic polyneuropathy.
FAP (familial amyloidotic polyneuropathy) is a systemic amyloid disease characterized by the formation of extracellular deposits of transthyretin. More than 80 single point mutations are associated with amyloidogenic behaviour and the onset of this fatal disease. It is believed that mutant forms of transthyretin lead to a decreased stability of the tetramer, which dissociates into monomers that...
متن کاملTransthyretin-related familial amyloidotic polyneuropathy.
Transthyretin-related familial amyloidotic polyneuropathy (FAP) is a fatal hereditary amyloidosis. Until 20 years ago, FAP was thought to be restricted to endemic occurrence in certain areas. However, owing to progress in biochemical and molecular genetic analyses, FAP is now believed to occur worldwide. As of today, reports of about 100 different points of single or double mutations, or a dele...
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Background Diflunisal is a well known FDA-registered commonly used NSAID therapy in the USA since the 1970’s. In Europe, the drug has been seldom authorised on a national basis with the next safety update report scheduled for 2025 (http://www.ema.europa.eu/ema/). Spain is one such country where commercial use has not been authorised, likely because of concerns on liver hypersensitivity and avai...
متن کاملPeritransplant gastrointestinal symptoms in familial amyloidotic polyneuropathy.
OBJECTIVES Gastrointestinal dysfunction is a common complication in familial amyloidotic polyneuropathy, and gastrointestinal symptoms are associated with a patient's nutritional status. The object of this study was to evaluate changes in peritransplant gastrointestinal symptoms and the nutritional status of familial amyloidotic polyneuropathy patients using the modified body mass index followi...
متن کاملGlial cells in familial amyloidotic polyneuropathy
INTRODUCTION Transthyretin V30M mutation is the most common variant leading to Familial Amyloidotic Polyneuropathy. In this genetic disorder, Transthyretin accumulates preferentially in the extracellular matrix of peripheral and autonomic nervous systems leading to cell death and dysfunction. Thus, knowledge regarding important biological systems for Transthyretin clearance might unravel novel ...
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ژورنال
عنوان ژورنال: Journal of Internal Medicine
سال: 2000
ISSN: 0954-6820,1365-2796
DOI: 10.1046/j.1365-2796.2000.00668.x