## Abstract Niemann‐Pick type C1 (NPC1) disease is an autosomal‐recessive cholesterol‐storage disorder characterized by liver dysfunction, hepatosplenomegaly, and progressive neurodegeneration. The NPC1 gene is expressed in every tissue of the body, with liver expressing the highest amounts of NPC1
Magnetization transfer contrast imaging in Niemann pick type C mouse liver
✍ Scribed by Jingyu Guo; Robert Erickson; Theodore Trouard; Jean-Philippe Galons; Robert Gillies
- Publisher
- John Wiley and Sons
- Year
- 2003
- Tongue
- English
- Weight
- 790 KB
- Volume
- 18
- Category
- Article
- ISSN
- 1053-1807
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✦ Synopsis
Abstract
Purpose
To investigate livers of mice afflicted with Niemann Pick type C (NP‐C) disease using magnetization transfer contrast (MTC) imaging and to test the hypothesis that the MT ratio reproducibly changes during disease progression.
Background
NP‐C is a heritable defect of lipid metabolism that results in the accumulation of unesterified cholesterol and gangliosides in virtually all cells. Symptoms predominate in brain and liver, which have high endogenous rates of lipid turnover. It is fatal to children, usually early in the second decade of life. Previous work has shown that the efficiency of magnetization transfer (MT) can be affected by cholesterol and collagen in tissues. The MT ratio (MTR) was calculated and compared during growth and therapy of diseased and control mice.
Results
Significant differences in the MTR were observed between livers of diseased and control mice. These ratios were consistent with collagen deposition associated with fibrosis, and not the accumulation of unesterified cholesterol in this organ.
Conclusion
These results indicate that MTC imaging may have clinical potential for monitoring progression and therapy in NP‐C disease. J. Magn. Reson. Imaging 2003;18:321–327. © 2003 Wiley‐Liss, Inc.
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