| RRC ID |
2807
|
| 著者 |
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.
|
| タイトル |
A transgenic rat with the human ATTR V30M: a novel tool for analyses of ATTR metabolisms.
|
| ジャーナル |
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.
|
| 巻・号 |
352(2)
|
| ページ |
299-304
|
| 公開日 |
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
|
| 引用数 |
18
|
|
WOS 分野
|
BIOPHYSICS
BIOCHEMISTRY & MOLECULAR BIOLOGY
|
| リソース情報 |
| ラット |
DA-Tg(Alb-TTR*V30M)7Jmsk(strainID=703)
DA-Tg(Alb-TTR*V30M)9Jmsk(strainID=705) |
