Sökning: "fibril composition"

Visar resultat 1 - 5 av 21 avhandlingar innehållade orden fibril composition.

  1. 1. The heart in hereditary transthyretin amyloidosis : clinical studies on the impact of amyloid fibril composition

    Författare :Björn Pilebro; Ole Suhr; Henning Mölgaard; Umeå universitet; []
    Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; Amyloidosis; Transthyretin; Cardiomyopathy; Echocardiography; Scintigraphy; Positron emission tomography; cardiovascular disease; hjärt- och kärlforskning; kardiologi; Cardiology; Clinical Physiology; klinisk fysiologi;

    Sammanfattning : Background Hereditary transthyretin amyloid (ATTRm) amyloidosis is a systemic disease mainly affecting the peripheral nervous system and the heart. The disease is inherited in an autosomal dominant manner with a varying penetrance. It is caused by mutations in the transthyretin (TTR) gene. Today more than 100 disease causing mutations are known. LÄS MER

  2. 2. Local variability in chemical and physical properties of spruce wood fibers

    Författare :Anna Bergander; KTH; []
    Nyckelord :cell walls; chemical composition; elastic constants; elastic strength; Fiber properties; Fibril angles; Mathematical models; Microscopy techniques; Picea Abies; Stiffness; Tensile properties; Wood polymers; Wood;

    Sammanfattning : Wood is well known to be a heterogeneous native materialwith large variations in its composite properties, includingvariations whose origins have not yet been completelyidentified. Traditionally, the macroscopic variations in woodhave received the most attention; for example, the variation indensity from the pith to the bark in trees. LÄS MER

  3. 3. Two Types of Fibrils in ATTR Amyloidosis : Implications for Clinical Phenotype and Treatment Outcome

    Författare :Elisabet Ihse; Per Westermark; Per Hammarström; Uppsala universitet; []
    Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; amyloid; transthyretin; familial amyloidotic polyneuropathy; TTRV30M; non-TTRV30M; wild-type; liver transplantation; cardiomyopathy; fibril composition; Biokemi; Biochemistry; Biology with specialization in Molecular Biology; Biologi med inriktning mot molekylärbiologi; Experimentell patologi; Experimental Pathology; Medical Biochemistry; Medicinsk biokemi; Molecular Biology; Molekylärbiologi; Pathology; Patologi;

    Sammanfattning : Systemic amyloidoses are a group of lethal diseases where proteins aggregate into fibrillar structures, called amyloid fibrils, that deposits throughout the body. Transthyretin (TTR) causes one type of amyloidosis, in which the aggregates mainly infiltrate nervous and cardiac tissue. LÄS MER

  4. 4. Cardiac function in hereditary transthyretin amyloidosis : an echocardiographic study

    Författare :Sandra Arvidsson; Per Lindqvist; Eva Nylander; Umeå universitet; []
    Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; Amyloid; echocardiography; ECG; HCM; fibril type; strain; ATTR; cardiomyopathy; speckle tracking;

    Sammanfattning : Background: Hereditary transthyretin amyloidosis (ATTR) is a lethal disease in which misfolded transthyretin (TTR) proteins accumulate as insoluble aggregates in tissues throughout the body. A common mutation is the exchange of valine to methionine at place 30 (TTR V30M), a form endemically found in the northern parts of Sweden. LÄS MER

  5. 5. α-Synuclein Fibril Formation and the Effects of Lipid Membranes

    Författare :Ricardo Gaspar; Fysikalisk kemi; []
    Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; NATURVETENSKAP; NATURAL SCIENCES; NATURVETENSKAP; NATURAL SCIENCES; Protein misfolding; alpha-synuclein; Parkinson s disease; microscopic rate constants; Thioflavin T; ganglioside lipids; Exosomes; protein-lipid co-aggregation; model membranes; monomer-dependent secondary nucleation;

    Sammanfattning : Amyloid protein aggregation results in major disturbances of cellular processes in humans. The most common amyloid-related disorders are Alzheimer´s disease and Parkinson´s disease. Parkinson´s disease is charaterized by the formation of protein-rich aggregates that are deposited in neurons, termed Lewy bodies and Lewy neurites. LÄS MER