Animal Models of Human Amyloidoses: Are Transgenic Mice Worth the Time and Trouble?

Overview

Journal FEBS Lett

Specialty Biochemistry

Date 2009 Jul 25

PMID 19627988

Citations 19

Authors

Joel N Buxbaum

Affiliations

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Abstract

The amyloidoses are the prototype gain of toxic function protein misfolding diseases. As such, several naturally occurring animal models and their inducible variants provided some of the first insights into these disorders of protein aggregation. With greater analytic knowledge and the increasing flexibility of transgenic and gene knockout technology, new models have been generated allowing the interrogation of phenomena that have not been approachable in more reductionist systems, i.e. behavioral readouts in the neurodegenerative diseases, interactions among organ systems in the transthyretin amyloidoses and taking pre-clinical therapeutic trials beyond cell culture. The current review describes the features of both transgenic and non-transgenic models and discusses issues that appear to be unresolved even when viewed in their organismal context.

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