Article: Co-precipitation molecules hemopexin and transferrin may be key molecules for fibrillogenesis in TTR V30M amyloidogenesis
Transgenic research. 2018 Feb., v. 27, no. 1
2018
Abstract: The disease model of familial amyloidotic polyneuropathy—7.2-hMet30 mice—manifests amyloid deposition that consists of a human amyloidogenic mutant transthyretin (TTR) (TTR V30M). Our previous study found amyloid deposits in 14 of 27 7.2-hMet30 mice at ... ...
| Abstract | The disease model of familial amyloidotic polyneuropathy—7.2-hMet30 mice—manifests amyloid deposition that consists of a human amyloidogenic mutant transthyretin (TTR) (TTR V30M). Our previous study found amyloid deposits in 14 of 27 7.2-hMet30 mice at 21–24 months of age. In addition, non-fibrillar TTR deposits were found in amyloid-negative 7.2hMet30 mice. These results suggested that TTR amyloidogenesis required not only mutant TTR but also an additional factor (or factors) as an etiologic molecule. To determine the differences in serum proteome in amyloid-positive and amyloid-negative mice in the 7.2-hMet30 model, we used proteomic analyses and studied serum samples obtained from these mice. Hemopexin (HPX) and transferrin (Tf) were detected in the serum samples from amyloid-positive mice and were also found in amyloid deposits via immunohistochemistry, but serum samples from amyloid-negative mice did not contain HPX and Tf. These two proteins were also not detected in non-fibrillar TTR deposits. In addition, in silico analyses suggested that HPX and Tf facilitate destabilization of TTR secondary structures and misfolding of TTR. These results suggest that HPX and Tf may be associated with TTR amyloidogenesis after fibrillogenesis in vivo. |
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| Keywords | amyloid ; blood serum ; coprecipitation ; disease models ; etiology ; genetic engineering ; humans ; immunohistochemistry ; mice ; mutants ; prealbumin ; proteome ; transferrin |
| Language | English |
| Dates of publication | 2018-02 |
| Size | p. 15-23. |
| Publishing place | Springer International Publishing |
| Document type | Article |
| ZDB-ID | 31620-9 |
| ISSN | 1573-9368 ; 0962-8819 |
| ISSN (online) | 1573-9368 |
| ISSN | 0962-8819 |
| DOI | 10.1007/s11248-017-0054-x |
| Database | NAL-Catalogue (AGRICOLA) |
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