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Title: Defective iron homeostasis in lysosomal storage diseases
Author: Chen, Chun-Wu
Awarding Body: University of Oxford
Current Institution: University of Oxford
Date of Award: 2013
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Niemann-Pick type Cl (NPC1) disease is a neurodegenerative lysosomal storage disorder characterized by the accumulation of multiple lipids in the late endosome/lysosomal system and reduced acidic store calcium levels. Since the lysosomal system is involved in regulating aspects of transition metal ion homeostasis and its intracellular compartmentalization, we have investigated whether there are metal ion metabolism defects and haematological abnormalities in NPC1 disease. We have identified multiple haematological changes, including decreased haematocrit, haemoglobin and mean corpuscular haemoglobin volume in mice.
Supervisor: Platt, Fran Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available
Keywords: Pharmacology ; Lysosomal storage diseases ; metal ; biomarker ; glycosphingolipids