Early Application of Auxiliary Partial Orthotopic Liver Transplantation in Murine Model of Wilson Disease. Issue 11 (November 2015)

• Record Type: Journal Article
• Authors: Cheng, Qi; He, Song-Qing; Gao, Dan; Lei, Biao; Long, Xin; Liang, Hui-Fang; Zhu, Peng; Jin, Jun-Fei; Tang, Bo; Tomlinson, Stephen; Wu, Zhi-Ying; Chen, Xiao-Ping
• Is Part Of: Transplantation. Volume 99:Issue 11(2015)
• Publication Date: 2015-11
• Subjects: Transplantation of organs, tissues, etc -- Periodicals; Transplantation immunology -- Periodicals; 617.95
• DOI: 10.1097/TP.0000000000000787
• Languages: English
• ISSNs: 0041-1337

Abstract

Abstract : Background: Liver transplantation (LT) is the only option of treatment for Wilson disease (WD) when chelation therapy fails, but it is limited due to the shortage of donor. Auxiliary partial orthotopic LT (APOLT) has been performed successfully in end-stage WD patients, which expands the donor pool. Methods: Atp7b −/− mice were used as experimental model of WD. Eight- and 20-week-old mice were used as different timepoints to perform APOLT. Serum copper, tissue copper, serum ceruloplasmin (CP), and liver histological examination were observed after operation. Results: Hepatic and serum copper levels in Atp7b −/− mice decreased after APOLT, and copper metabolism disorder of WD mice was relieved at both early and late stages. The progression of pathology in the native liver was delayed only when transplantation was performed at an early stage. Conclusions: Auxiliary partial orthotopic LT can significantly improve copper metabolism disorder in the Atp7b −/− mice, and early transplantation may prevent the disease progression. Abstract : Auxiliary partial orthotopic liver transplantation (APOLT) has been performed successfully in end-stage Wilson Disease (WD) patients. Cheng et al describe a surgical model of APOLT in Atp7b-/- mice lacking the copper transporting P-type adenosine triphosphatase that is causative for WD.