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Urinary copper elevation in a mouse model of Wilson's disease is a regulated process to specifically decrease the

Lawrence W Gray1, Fangyu Peng, Shannon A Molloy

  • 1Department of Physiology, Johns Hopkins University, School of Medicine, Baltimore, Maryland, USA.

Plos One
|July 18, 2012
PubMed
Summary
This summary is machine-generated.

Wilson

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Area of Science:

  • Biochemistry
  • Physiology
  • Medical Science

Background:

  • Copper homeostasis is critical for health, regulated mainly by the liver.
  • Wilson's disease (WD) involves disrupted copper excretion due to ATP7B gene mutations, leading to liver copper overload.
  • Elevated urinary copper is a diagnostic marker for WD, but its underlying mechanism remains unclear.

Purpose of the Study:

  • To elucidate the mechanism of elevated urinary copper in Wilson's disease.
  • To investigate the role of copper transporters and novel molecules in copper excretion.
  • To understand the body's regulatory processes for copper export.

Main Methods:

  • Longitudinal study using Positron Emission Tomography-Computed Tomography (PET-CT) imaging in Atp7b(-/-) mice.
  • Metal analysis using atomic absorption spectroscopy.
  • Characterization of copper-binding molecules in urine and serum.

Main Results:

  • A decrease in hepatic copper accumulation capacity correlated with increased urinary copper in Atp7b(-/-) mice.
  • Urinary copper elevation was linked to decreased hepatic Ctr1 expression and the appearance of a 2 kDa Small Copper Carrier (SCC) in urine.
  • SCC, identified as a serum copper carrier, was also elevated in liver-specific Ctr1 knockouts, indicating its role in copper excretion.

Conclusions:

  • Urinary copper elevation in WD is a regulated process involving SCC, not solely cell necrosis.
  • Down-regulation of hepatic Ctr1 shifts copper excretion to the kidneys via SCC when liver function is compromised.
  • Understanding urinary copper excretion mechanisms can improve WD diagnosis and monitoring.