Correction: Penicillamine Increases Free Copper and Enhances Oxidative Stress in the Brain of Toxic Milk Mice

Ding-Bang Chen,Fu-Rong Li,Xun-Hua Li,Xiao-Pu Lin,Wei Zhang,Li Feng,Xiu-Ling Liang,Thomas Langmann

doi:10.1371/annotation/e57c1988-71fd-4809-8d84-aff56e60b2c7

Abstract

The following information was missing from the Funding section: This study was also supported by the National Natural Science Foundation of China grant #81171070.

Highlights

Wilson disease (WD) is an autosomal recessive disease arising from a mutation in the ATP7B gene, which results in the failure of hepatocytes to excrete copper into bile, leading to the hepatic copper accumulation and cell injury
The pivotal role of penicillamine in the initial treatment for WD patients with neurologic symptoms has been a matter of debate for the risk of neurological worsening for the past three decades. 10%–50% of patients neurologically worsened by PA and 50% of them never recovered to the level before treatment [2]
Copper concentrations The average serum free copper concentration detected by ultrafiltration method was about 8.9% of serum copper concentration, the sum of free copper and protein-bound copper, in control group of tx mice,while in derived from the wild-type (DL) mice it was 1.9%

Summary

Introduction

Wilson disease (WD) is an autosomal recessive disease arising from a mutation in the ATP7B gene, which results in the failure of hepatocytes to excrete copper into bile, leading to the hepatic copper accumulation and cell injury. Copper incorporation into the protein ceruloplasmin (Cp) is impaired by dysfunction of ATP7B, leading to the reduced circulating holoceruloplasmin. The treatment for WD patients involves reversing the positive copper balance. The pivotal role of penicillamine in the initial treatment for WD patients with neurologic symptoms has been a matter of debate for the risk of neurological worsening for the past three decades. 10%–50% of patients neurologically worsened by PA and 50% of them never recovered to the level before treatment [2]. Trientine shares the propensity of penicillamine to deteriorate neurologic symptoms (about 25%) [3]

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