Global ETD Search
Search theses and dissertations gathered from participating repositories worldwide. Every result links back to the library that holds it. No account is needed.
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Showing 1 to 20 of 43 for “"amyloidosis"”.
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Structural determinants of cardiac light chain amyloidosis
Light chain amyloidosis (AL) is an acute systemic amyloid disease in which the overexpression and misfolding of immunoglobulin light chains leads to the formation of insoluble fibrils at critical organs in the body. The typical prognosis for AL patients is extremely severe—usually less than three …
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Domain swapping as a molecular mechanism in amyloidosis
Amyloidosis is a group of diseases characterized by a change in protein conformation resulting in aggregation and tissue deposition of amyloid fibrils. One variant of cystatin C, L68Q cystatin C, is highly amyloidogenic and persons carrying the corresponding gene suffer from massive cerebral …
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Transthyretin Amyloidosis: Proteolytic cleavage accelerates G53A TTR misfolding and aggregation
… peptide bond and also associated with TTR amyloidosis, was investigated in thepresence and absence of proteolytic agent, trypsin. Fragmented G53A TTR misfolded and aggregated via a similar mechanism as full-length TTR, but at a faster rate. Similar morphology was exhibited by fragmented and …
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Liver transplantation for transthyretin amyloidosis: experience of a single center in Italy
… the only definitive treatment for transthyretin amyloidosis, with an excellent 5-year survival in endemic countries where the Met30 mutation is predominant. We report our experience of liver transplantation for transthyretin amyloidosis. We reviewed the clinical records of 17 transplanted …
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High-resolution characterization of structural changes involved in prion diseases and dialysis-related amyloidosis
Proteinaggregation ist die Ursache vieler Krankheiten, wie Diabetes Mellitus Typ 2, Parkinsonsche, Alzheimersche und Huntingtonsche Krankheit, spongiforme Encephalopathien, Stauungsinsuffizienz und Dialyse-assoziierte Amyloidose. All diesen Störungen liegt eine Proteinfehlfaltung zugrunde, die zur …
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Immunotherapy for Alzheimer’s Disease: IVIg Delivery to the Hippocampus in a Mouse Model of Amyloidosis
… pathology in the TgCRND8 (Tg) mouse model of amyloidosis. In 3-month-old Tg mice, we found that the hippocampal bioavailability of IVIg was increased by 7-fold with FUS. Within one week, IVIg was cleared from the hippocampus. We discovered that two weekly treatments of IVIg with FUS promoted …
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Determinants and Monitoring of Response to Disease-Modifying Therapy for Transthyretin Amyloidosis Cardiomyopathy: The ATTR-CM Therapy Study
Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is a heart muscle disease characterized by the accumulation of misfolded transthyretin proteins as amyloid plaques in the myocardial interstitium. In 2020, tafamidis, a medication inhibiting the misfolding of transthyretin, received Health Canada's …
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A fluidized immunoadsorption device for removing beta-2-microglobulin from whole blood : a potential treatment for dialysis-related amyloidosis
Dialysis-related amyloidosis (DRA) is a frequent complication of end-stage renal disease that has been associated with the accumulation of 2-microglobulin (2m). Excluding transplantation, existing kidney replacement technologies are believed to remove insufficient quantities of P2m for the …
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Role of Hepatic Lipoprotein Receptor-related Protein 1 (LRP-1) on Amyloid Beta Pathology in a Mouse Model of Amyloidosis
… 1 on amyloid beta pathology in a mouse model of amyloidosis.</p> <p>Alzheimer’s disease (AD) is a chronic neurodegenerative disease caused by an abundance of extracellular neuritic plaques containing enormous amounts of amyloid beta (Aβ) and excessive numbers of tau-rich neurofibrillary tangles. …
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MRI determined tissue characterization of myocardial infiltration and fibrosis in cardiomyopathy
… processes that cause cardiomyopathies. Amyloidosis comprises a group of diseases that are characterized by the extracellular deposition of insoluble fibrillar proteins in organs. The deposition of amyloid material in the heart leads to presentations of congestive heart failure mainly of …
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Molecular level characterisation of apolipoprotein A-I aggregation leading to fibrils comprising of both α-helical and β-sheet structures
Amyloidosis is defined as the misfolding of native proteins into insoluble fibrils that are deposited within tissues and extracellular organs. 30+ structurally and sequentially unrelated proteins have the ability to form amyloid aggregates, all of which contain characteristic features. ApoA-I, the …
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An investigation into the effects of inorganic element status on the accumulation and deposition of amyloid in various diseases.
… (β2M) deposition in dialysis related amyloidosis. The major factor for amyloidosis appears to be an increase in the production of amyloidogenic proteins, resulting in the amyloid β sheet deposits. It is clear that conformational changes in amyloidogenic proteins lead to fibril …
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Mass spectrometry studies of immunoglobulins
… space; this pathologic process, called primary amyloidosis or Ig LC amyloidosis (AL) causes problems to multiple organs during the course of the disease. Post-translational modifications (PTMs), which remain to be explored, are likely an important factor affecting the formation of AL fibrils. In …
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Measurement of urinary glycosaminoglycans in dogs
… with glomerulonephritis (GN), and those with amyloidosis thus potentially allowing differentiation without a renal biopsy. Aims of this study were to validate a simple spectrophotometric assay used to measure canine urinary GAGs, establish a normal reference range, and determine optimal …
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AMILOIDOSE - DEMANDAS E PERFIL FONOAUDIOLÓGICO: Revisão de literatura
Introduction: Amyloidosis refers to a set of diseases in which protein fragments, folded in a highly stable configuration, are pathogenically deposited in the extracellular space of organs and tissues as insoluble fibrils. The reason these fibrils accumulate is not yet clearly elucidated. Once …
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SERUM AMYLOID A IN RUMINANTS: DIAGNOSTIC VALUE AND FOOD CONTAMINATION ASSESSMENT
… be isolated from tissues of bovine with clinical amyloidosis was investigated. We also investigated if AA fibrils present in milk can be then found in cheese after caseification, i.e. if the process of ripeining can degrade the AA fibrils. In bovine, SAA was identified as potential marker of …
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The APOE Pathway as a Modulator of Amyloid Pathology in Alzheimer's Disease Models
… first used microRNA-33 knockout mice within an amyloidosis mouse model to determine if increased ABCA1 and apoE lipidation affect amyloid pathology. We demonstrate that deleting microRNA-33 reduced Aβ levels and plaque deposition. Through our multi-omics approach, we identified that microRNA-33 …
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Protein mis-folding and human disease
… fibrils of<br/>misfolded proteins, which cause amyloidosis in humans. SAP not only stabilizes<br/>amyloid fibrils but also protects them from proteolytic and cell mediated<br/>degradation. SAP has been co-crystallized with three different aminoalkyl<br/>phosphonates that bind at the amyloid …
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Targeted magnetic nanoparticles for remote manipulation of protein aggregation
… targeting scheme has potential as a therapy for amyloidosis and as a minimally invasive tool for analyzing and controlling protein aggregation.
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