Imaging of changes in copper trafficking and redistribution in a mouse model of Niemann-Pick C disease using positron emission tomography View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2019-04

AUTHORS

Julia Baguña Torres, Zilin Yu, Jayanta Bordoloi, Kavitha Sunassee, David Smith, Claire Smith, Oscar Chen, Rupert Purchase, Karin Tuschl, John Spencer, Frances Platt, Philip J. Blower

ABSTRACT

Niemann-Pick C disease (NPC) is an autosomal recessive lysosomal storage disorder resulting from mutations in the NPC1 (95% of cases) or NPC2 genes. Disturbance of copper homeostasis has been reported in NPC1 disease. In this study we have used whole-body positron emission tomography (PET) and brain electronic autoradiography with copper-64 (64Cu), in the form of the copper(II) bis(thiosemicarbazonato) complex 64Cu-GTSM, to image short-term changes in copper trafficking after intravenous injection in a transgenic mouse model of NPC1 disease. 64Cu-GTSM is taken up in all tissues and dissociates rapidly inside cells, allowing monitoring of the subsequent efflux and redistribution of 64Cu from all tissues. Significantly enhanced retention of 64Cu radioactivity was observed in brain, lungs and blood at 15 h post-injection in symptomatic Npc1-/- transgenic mice compared to wildtype controls. The enhanced retention of 64Cu in brain was confirmed by electronic autoradiography, particularly in the midbrain, thalamus, medulla and pons regions. Positron emission tomography imaging with 64Cu in selected chemical forms could be a useful diagnostic and research tool for the management and understanding of NPC1 disease. More... »

PAGES

293-306

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s10534-019-00185-5

DOI

http://dx.doi.org/10.1007/s10534-019-00185-5

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1112607093

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/30847690


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44 schema:description Niemann-Pick C disease (NPC) is an autosomal recessive lysosomal storage disorder resulting from mutations in the NPC1 (95% of cases) or NPC2 genes. Disturbance of copper homeostasis has been reported in NPC1 disease. In this study we have used whole-body positron emission tomography (PET) and brain electronic autoradiography with copper-64 (<sup>64</sup>Cu), in the form of the copper(II) bis(thiosemicarbazonato) complex <sup>64</sup>Cu-GTSM, to image short-term changes in copper trafficking after intravenous injection in a transgenic mouse model of NPC1 disease. <sup>64</sup>Cu-GTSM is taken up in all tissues and dissociates rapidly inside cells, allowing monitoring of the subsequent efflux and redistribution of <sup>64</sup>Cu from all tissues. Significantly enhanced retention of <sup>64</sup>Cu radioactivity was observed in brain, lungs and blood at 15 h post-injection in symptomatic Npc1<sup>-/-</sup> transgenic mice compared to wildtype controls. The enhanced retention of <sup>64</sup>Cu in brain was confirmed by electronic autoradiography, particularly in the midbrain, thalamus, medulla and pons regions. Positron emission tomography imaging with <sup>64</sup>Cu in selected chemical forms could be a useful diagnostic and research tool for the management and understanding of NPC1 disease.
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