PMID- 20930032 OWN - NLM STAT- MEDLINE DCOM- 20110222 LR - 20101108 IS - 1758-1001 (Electronic) IS - 0004-5632 (Linking) VI - 47 IP - Pt 6 DP - 2010 Nov TI - Effect of recipient-derived cells on the progression of familial amyloidotic polyneuropathy after liver transplantation: a retrospective study. PG - 529-34 LID - 10.1258/acb.2010.010156 [doi] AB - BACKGROUND: Some familial amyloidotic polyneuropathy (FAP) patients show the post-transplant progression of the clinical symptoms. Although the presence of recipient-derived cells in transplanted livers has been reported, no studies investigating the functional significance of this post-transplant chimerism in transplanted FAP patients were performed. The aims of this study were to evaluate amyloidogenic transthyretin (ATTR) production of recipient-derived cells and the relationship between the protein from recipient-derived cells and the progression of FAP symptoms after liver transplantation (LT). METHODS: Seven FAP ATTR Val30Met patients who underwent LT were included in this study. In one male patient with sex-mismatched donor, fluorescence in situ hybridization (FISH) method was performed on a liver biopsy sample using DNA probes for visualizing X and Y chromosomes to detect the recipient-derived cells. In three patients including the FISH-analysed patient, ATTR mRNA expression in transplanted livers was evaluated by the polymerase chain reaction (PCR)-restriction fragment length polymorphism method and realtime quantitative reverse transcription-PCR. In five of the seven patients, ATTR in serum protein expression was measured by mass spectrometry. RESULTS: One FAP patient has 3.1% recipient-derived cells in the transplanted liver. The ATTR mRNA was not expressed in any of the three transplanted livers. The ATTR was not detected in any sera of the sampled patients. CONCLUSION: Although the FAP patient had recipient-derived cells in the transplanted liver, the recipient-derived cells did not contribute to the production of ATTR in our specific case. The effect of recipient-derived cells on the post-transplant progression of FAP symptoms may be negligible. FAU - Ohya, Yuki AU - Ohya Y AD - Department of Transplantation and Pediatric Surgery, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto, Japan. FAU - Jono, Hirofumi AU - Jono H FAU - Nakamura, Masaaki AU - Nakamura M FAU - Hayashida, Shintaro AU - Hayashida S FAU - Ueda, Mitsuharu AU - Ueda M FAU - Obayashi, Konen AU - Obayashi K FAU - Misumi, Shogo AU - Misumi S FAU - Asonuma, Katsuhiro AU - Asonuma K FAU - Ando, Yukio AU - Ando Y FAU - Inomata, Yukihiro AU - Inomata Y LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20101007 PL - England TA - Ann Clin Biochem JT - Annals of clinical biochemistry JID - 0324055 RN - 0 (Amyloidogenic Proteins) RN - 0 (Prealbumin) SB - IM MH - Adult MH - Amyloid Neuropathies, Familial/*genetics/metabolism/*therapy MH - Amyloidogenic Proteins/genetics MH - Chromosomes, Human, X/genetics MH - Chromosomes, Human, Y/genetics MH - Female MH - Humans MH - In Situ Hybridization, Fluorescence MH - Liver/cytology/metabolism MH - *Liver Transplantation/adverse effects MH - Male MH - Middle Aged MH - Polymerase Chain Reaction MH - Polymorphism, Restriction Fragment Length MH - Prealbumin/genetics MH - Retrospective Studies MH - Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization EDAT- 2010/10/12 06:00 MHDA- 2011/02/23 06:00 CRDT- 2010/10/09 06:00 PHST- 2010/10/09 06:00 [entrez] PHST- 2010/10/12 06:00 [pubmed] PHST- 2011/02/23 06:00 [medline] AID - acb.2010.010156 [pii] AID - 10.1258/acb.2010.010156 [doi] PST - ppublish SO - Ann Clin Biochem. 2010 Nov;47(Pt 6):529-34. doi: 10.1258/acb.2010.010156. Epub 2010 Oct 7.