Navegando por Palavras-chave "amyloidosis"
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- ItemSomente MetadadadosCommentary: Evidence for human transmission of amyloid-beta pathology and cerebral amyloid angiopathy(Frontiers Media Sa, 2016) Souza, Isis Nem De Oliveira; De-Souza, Evandro A. [UNIFESP]
- ItemAcesso aberto (Open Access)Commentary: Evidence for human transmission of amyloid-beta pathology and cerebral amyloid angiopathy(Frontiers Media Sa, 2016) Souza, Isis Nem De Oliveira; De-Souza, Evandro A. [UNIFESP]
- ItemSomente MetadadadosEvaluation of Aggregation and Fibril Formation Propensity of Peptides Involved in Amyloid Diseases(Bentham Science Publ Ltd, 2017) Malavolta, Luciana; Chinarelli, Ricardo Lobato; Sobral, Danielle Vieira; Nakaie, Clovis R. [UNIFESP]Background: Amyloidosis is defined as a generic term given to a series of proteins/polypeptides in the form of amyloid fibrils that are deposited in the tissues and give rise to a set of clinical disorders. Objectives: This work developed an approach to first examine chain association propensities of several amyloidogenic peptides: SNNFGAILSS from the islet amyloid polypeptide (coded IAPP), NAGDVAFV from the protein responsible for corneal amyloidosis (coded Lactoferrin), and (1-42) beta-amyloid (coded Amyloid). Methods: Fmoc-synthesis protocol was applied for the synthesis of IAPP and Lactoferrin whereas Amyloid was synthesized through the Boc-chemistry as early detailed. Results and Conclusion: The fluorescence and light scattering experiments results indicated that Amyloid revealed a surprising reduction in the aggregation process as a function of time (decrease of about 20-30% in 3 days) through both methods. In contrast, the aggregation intensity of IAPP increased around 35% after 3 days via a light scattering procedure. These findings are very relevant for interpretation of the aggregation phenomenon of amyloidogenic peptides. The final part of this work proposed rules for dissolution of aggregated structures based on the Lewis acid and Lewis base properties of solvents. Very low solubility values (6 to 15%) were measured for peptides in water but with increased to around 90% in strong nucleophilic or strong electrophilic organic solvents. However, care should be taken when strong nucleophilic solvents such as DMSO are mixed with the strong electrophilic such as water. Both solvent molecules tend to attract each other rather than to dissolve peptide chains thus lowering the capacity of this type of solution for fibril dissolution.
- ItemAcesso aberto (Open Access)Macroglossia como primeira manifestação clínica da amiloidose primária(Sociedade Brasileira de Reumatologia, 2007-02-01) Alambert, Carla De Oliveira; Sarpi, Maíra De Oliveira; Dedivitis, Rogério Aparecido [UNIFESP]; Alambert, Paulo Augusto; Sementilli, Ângelo [UNIFESP]; Arantes, Luciana Pierotti; Universidade Metropolitana de Santos; Centro Universitário Lusíada; Universidade Federal de São Paulo (UNIFESP); Sociedade Brasileira de Reumatologia; Hospital Ana Costa Clínica MédicaAmyloidosis is a rare disease caused by pathological deposit of an amyloid extracellular proteinaceus material. We report a case of a 50-year-old man with history of lips and tongue swelling associated to periorbital ecchimosys for 6 months. At the physical examination an important macroglossia was observed. Complementary tests were accomplished, and the pathological examination of the tongue biopsy confirmed the diagnosis. The patient was treated with prednisone and melphalan with an unsatisfactory response. Along the follow up the patient developed heart failure, and died 7 months after diagnosis of pulmonary infection and sepsis.
- ItemSomente MetadadadosThe most recent advances in pathophysiology and management of tumour necrosis factor receptor associated periodic syndrome (TRAPS): personal experience and literature review(Clinical & Exper Rheumatology, 2013-05-01) Magnotti, F.; Vitale, A.; Rigante, D.; Lucherini, O. M.; Cimaz, R.; Muscari, I.; Faria, Atila Granados Afonso de [UNIFESP]; Frediani, B.; Galeazzi, M.; Cantarini, L.; Univ Siena; Univ Cattolica Sacro Cuore; Anna Meyer Childrens Hosp; Universidade Federal de São Paulo (UNIFESP)Tumour necrosis factor-receptor associated periodic syndrome (TRAPS) is a rare autosomal dominant autoinflammatory disorder characterised by recurrent episodes of long-lasting fever and inflammation in different regions of the body, as musculo-skeletal system, skin, gastrointestinal tube, serosal membranes and eye. Inflammatory attacks usually start in paediatric age with initial corticosteroid-responsiveness. Most reported cases of TRAPS involve patients of European ancestry and diagnosis can be formulated by the combination of genetic analysis and a compatible phenotype. Its prognosis is strictly dependent on the appearance of amyloidosis, secondary to uncontrolled relapsing inflammation. Thanks to a better understanding of its pathogenesis, the disease is now managed with anti-interleukin (IL)-1 antagonists, rather than corticosteroids or tumour necrosis factor (TNF) inhibitors. The aim of this review is to describe the current understanding and advances of TRAPS genetic basis, pathogenesis and management options by integrating the most recent data in the medical literature.