Positron emission tomography of copper metabolism in the Atp7b-/- knock-out mouse model of Wilson's disease

Mol Imaging Biol. 2012 Feb;14(1):70-8. doi: 10.1007/s11307-011-0476-4.

Abstract

Purpose: This study aims to determine feasibility and utility of copper-64(II) chloride (⁶⁴CuCl₂) as a tracer for positron emission tomography (PET) of copper metabolism imbalance in human Wilson's disease (WD).

Procedures: Atp7b⁻/⁻ mice, a mouse model of human WD, were injected with ⁶⁴CuCl₂ intravenously and subjected to PET scanning using a hybrid PET-CT (computerized tomography) scanner, with the wild-type C57BL mice as a normal control. Quantitative PET analysis was performed to determine biodistribution of ⁶⁴Cu radioactivity and radiation dosimetry estimates of ⁶⁴Cu were calculated for PET of copper metabolism in humans.

Results: Dynamic PET analysis revealed increased accumulation and markedly reduced clearance of ⁶⁴Cu from the liver of the Atp7b⁻/⁻ mice, compared to hepatic uptake and clearance of ⁶⁴Cu in the wild-type C57BL mice. Kinetics of copper clearance and retention was also altered for kidneys, heart, and lungs in the Atp7b/⁻ mice. Based on biodistribution of ⁶⁴Cu in wild-type C57BL mice, radiation dosimetry estimates of ⁶⁴Cu in normal human subjects were obtained, showing an effective dose (ED) of 32.2 μ (micro)Sv/MBq (weighted dose over 22 organs) and the small intestine as the critical organ for radiation dose (61 μGy/MBq for males and 69 μGy/MBq for females). Radiation dosimetry estimates for the patients with WD, based on biodistribution of ⁶⁴Cu in the Atp7b⁻/⁻ mice, showed a similar ED of 32.8 μ (micro)Sv/MBq (p = 0.53), with the liver as the critical organ for radiation dose (120 μSv/MBq for male and 161 μSv/MBq for female).

Conclusions: Quantitative PET analysis demonstrates abnormal copper metabolism in the mouse model of WD with improved time-resolution. Human radiation dosimetry estimates obtained in this preclinical study encourage direct radiation dosimetry of ⁶⁴CuCl₂ in human subjects. The results suggest feasibility of utilizing ⁶⁴CuCl₂ as a tracer for noninvasive assessment of copper metabolism in WD with PET.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenosine Triphosphatases / deficiency*
  • Adenosine Triphosphatases / genetics
  • Adenosine Triphosphatases / metabolism
  • Animals
  • Cation Transport Proteins / deficiency*
  • Cation Transport Proteins / genetics
  • Cation Transport Proteins / metabolism
  • Copper / metabolism*
  • Copper Radioisotopes*
  • Copper-Transporting ATPases
  • Hepatolenticular Degeneration / genetics
  • Hepatolenticular Degeneration / metabolism*
  • Humans
  • Kinetics
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Positron-Emission Tomography / methods*
  • Radiometry
  • Tissue Distribution

Substances

  • Atp7a protein, mouse
  • Cation Transport Proteins
  • Copper Radioisotopes
  • Copper
  • Adenosine Triphosphatases
  • Copper-Transporting ATPases
  • cupric chloride