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Springer Der intrazelluläre Proteintransport bei Erkrankungen im Kindesalter

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Description

Die Arbeit faßt die bisherigen Erkenntnisse über intrazelluläre Transportprozesse und ihre klinische Bedeutung zusammen, beschreibt die Methoden zur Untersuchung intrazellulärer Transportprozesse und präsentiert neue Ergebnisse aus eigenen Forschungsarbeiten über Krankheiten mit Veränderungen des intrazellulären Proteintransportes wie Zöliakie, Ornithin-Transcarbamylase-Mangel und Morbus Gaucher. From the Back Cover Der intrazelluläre Proteintransport stellt einen wichtigen Faktor zahlreicher Zellfunktionen dar. Bei vielen Erkrankungen sind intrazelluläre Transportvorgänge verändert. Durch Transportdefekte kann die Diagnostik, insbesondere von angeborenen Stoffwechselerkrankungen erschwert sein. Verschiedene Untersuchungsmethoden einschließlich der Kryoultramikrotomie und Immungold-Methode werden vorgestellt, die geeignet sind, Defekte oder Veränderungen des intrazellulären Proteintransportes festzustellen. Am Beispiel der thyreoidalen Peroxidase, des Hemagglutinins und der lysomalen Membranproteine werden physiologische Transportvorgänge der Exozytose und Endozytose dargestellt. Beim Ornithin-Transcarbamylase-Mangel und Carbamylphosphat-Synthetase-Mangel, zwei Harnstoffzyklus-Defekten, steht der mitochondriale Importmechanismus im Vordergrund der zellbiologischen Pathophysiologie. Auf die Bedeutung des biosynthetischen Transportes wird beim Morbus Gaucher hingewiesen. Bei der Zöliakie werden pathognomische Transportprozesse innerhalb des Endozytose-Apparates von Enterozyten vorgestellt.

Product Specifications

Format
Paperback
Domain
Amazon UK
Release Date
01 January 1997
Listed Since
21 February 2011

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