title: Specific pathogen free conditions prevent transthyretin amyloidosis in mouse models.
abstract: Transthyretin (TTR) associated amyloidosis is an autosomal dominant disorder characterized by peripheral and autonomic neuropathy. Both genetic and environmental factors are thought to be involved in development of TTR associated amyloidosis. Previously, we demonstrated that amyloid deposition was observed in various tissues of transgenic mouse lines carrying a human mutant TTR (Met30) gene. To analyze the influence of environmental factors on TTR amyloidosis, these amyloidogenic transgenic mouse models were kept under conventional (CV) or specific pathogen free (SPF) conditions. Although the serum levels of Met30 for mice housed in the CV and SPF conditions were similar, amyloid deposition was observed in CV conditions, but not in SPF conditions. In addition, the extent of amyloid deposition in transgenic mice was dependent on duration kept under CV conditions. There were significant differences in proportion of amyloid deposition in several tissues between CV and SPF conditions. Maintenance of these mice at 30 degrees C did not induce amyloid deposition in SPF conditions. These results suggest that the SPF conditions can completely prevent amyloid deposition, and that environmental factors can affect the onset and progression even in a single gene disorder.
authors: Institute of Molecular Embryology and Genetics, Kumamoto University, 2-2-1 Honjo, Kumamoto, 860-0811, Japan.
authors: Seiya
authors: S
authors: Inoue
authors: Mika
authors: M
authors: Ohta
authors: Zhenghua
authors: Z
authors: Li
authors: Gang
authors: G
authors: Zhao
authors: Yutaka
authors: Y
authors: Takaoka
authors: Naomi
authors: N
authors: Sakashita
authors: Kazuhisa
authors: K
authors: Miyakawa
authors: Koji
authors: K
authors: Takada
authors: Hajime
authors: H
authors: Tei
authors: Misao
authors: M
authors: Suzuki
authors: Michio
authors: M
authors: Masuoka
authors: Yoshiyuki
authors: Y
authors: Sakaki
authors: Kiyoshi
authors: K
authors: Takahashi
authors: Ken-Ichi
authors: K
authors: Yamamura
mesh_terms: D000686:Amyloidosis / Q000378:metabolism / Q000517:prevention & control*; D000818:Animals; D015153:Blotting, Western; D004195:Disease Models, Animal; D051379:Mice; D016133:Polymerase Chain Reaction; D011228:Prealbumin / Q000378:metabolism*
chemical_list: D011228:Prealbumin