Reference - Detail
| RRC ID | 2807 |
|---|---|
| Author | Ueda M, Ando Y, Hakamata Y, Nakamura M, Yamashita T, Obayashi K, Himeno S, Inoue S, Sato Y, Kaneko T, Takamune N, Misumi S, Shoji S, Uchino M, Kobayashi E. |
| Title | A transgenic rat with the human ATTR V30M: a novel tool for analyses of ATTR metabolisms. |
| Journal | Biochem Biophys Res Commun |
| Abstract |
Amyloidogenic transthyretin (ATTR) is the pathogenic protein of familial amyloidotic polyneuropathy (FAP). To establish a tool for analyses of ATTR metabolisms including after liver transplantations, we developed a transgenic rat model expressing human ATTR V30M and confirmed expressions of human ATTR V30M in various tissues. Mass spectrometry for purified TTR revealed that rat intrinsic TTR and human ATTR V30M formed tetramers. Congo red staining and immunohistochemistry revealed that nonfibrillar deposits of human ATTR V30M, but not amyloid deposits, were detected in the gastrointestinal tracts of the transgenic rats. At 24h after liver transplantation, serum human ATTR V30M levels in transgenic rats that received livers from normal rats became lower than detectable levels. These results thus suggest that this transgenic rat may be a useful animal model which analyzes the metabolism of human ATTR V30M including liver transplantation studies. |
| Volume | 352(2) |
| Pages | 299-304 |
| Published | 2007-1-12 |
| DOI | 10.1016/j.bbrc.2006.11.045 |
| PII | S0006-291X(06)02456-9 |
| PMID | 17126291 |
| MeSH | Amyloid Neuropathies, Familial / genetics* Amyloid Neuropathies, Familial / metabolism Animals Animals, Genetically Modified / genetics Animals, Genetically Modified / metabolism* Animals, Genetically Modified / surgery Disease Models, Animal* Humans Liver Transplantation Prealbumin / metabolism* Protein Engineering / methods Rats / genetics* Rats / metabolism* Rats / surgery Recombinant Proteins / metabolism |
| IF | 2.985 |
| Times Cited | 18 |
| WOS Category | BIOPHYSICS BIOCHEMISTRY & MOLECULAR BIOLOGY |
| Altmetric score |
オルトメトリクス指標項目
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| The most frequently cited source | Patent(IFI CLAIMS) |
| Total number of mentions | 27 |
| Altmetric score changes over past 6months | 0.0 |
| Resource | |
| Rats | DA-Tg(Alb-TTR*V30M)7Jmsk(strainID=703) DA-Tg(Alb-TTR*V30M)9Jmsk(strainID=705) |