title: Early Application of Auxiliary Partial Orthotopic Liver Transplantation in Murine Model of Wilson Disease.
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
  Atp7bmice 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.
authors.affiliation: 1 Hepatic Surgery Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. 2 Department of Hepatopancreatobiliary Surgery, the affiliated hospital of Guilin Medical University, Guilin, China. 3 Department of Microbiology and Immunology, Children's Research Institute, Medical University of South Carolina, Charleston, SC. 4 Department of Neurology and Research center of Neurology in Second Affiliated Hospital and the Collaborative Innovation Center for Brain Science, Zhejiang University School of Medicine, Hangzhou, China. 5 Department of Neurology and Institute of Neurology, Huashan Hospital, Institutes of Brain Science and State Key Laboratory of Medical Neurobiology, Shanghai Medical College, Fudan University, Shanghai, China.
authors.forename: Qi
authors.initials: Q
authors.lastname: Cheng
authors.forename: Song-Qing
authors.initials: SQ
authors.lastname: He
authors.forename: Dan
authors.initials: D
authors.lastname: Gao
authors.forename: Biao
authors.initials: B
authors.lastname: Lei
authors.forename: Xin
authors.initials: X
authors.lastname: Long
authors.forename: Hui-Fang
authors.initials: HF
authors.lastname: Liang
authors.forename: Peng
authors.initials: P
authors.lastname: Zhu
authors.forename: Jun-Fei
authors.initials: JF
authors.lastname: Jin
authors.forename: Bo
authors.initials: B
authors.lastname: Tang
authors.forename: Stephen
authors.initials: S
authors.lastname: Tomlinson
authors.forename: Zhi-Ying
authors.initials: ZY
authors.lastname: Wu
authors.forename: Xiao-Ping
authors.initials: XP
authors.lastname: Chen
mesh_terms: D000251:Adenosine Triphosphatases / Q000172:deficiency / Q000235:genetics; D000818:Animals; D015415:Biomarkers / Q000097:blood; D027682:Cation Transport Proteins / Q000172:deficiency / Q000235:genetics; D002570:Ceruloplasmin / Q000378:metabolism; D003300:Copper / Q000097:blood; D000073840:Copper-Transporting ATPases; D004195:Disease Models, Animal; D018450:Disease Progression; D005240:Feasibility Studies; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D008099:Liver / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D013997:Time Factors
chemical_list: C486201:Atp7a protein, mouse; D015415:Biomarkers; D027682:Cation Transport Proteins; D003300:Copper; D002570:Ceruloplasmin; D000251:Adenosine Triphosphatases; D000073840:Copper-Transporting ATPases