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Article Dans Une Revue Amyloid: The Journal of Protein Folding Disorders Année : 2011

Strategies to model AL amyloidosis in mice.

Résumé

Monoclonal immunoglobulin (Ig) deposition in AL amyloidosis is a severe complication of lymphoproliferative disorders. Research on this disease suffers from the lack of animal models, as they could allow for the testing of new innovative therapeutic strategies. We are trying to develop a transgenic animal model for this disease by overexpressing Ig light chain (LC) sequences cloned from AL amyloidosis patients. After several unsuccessful attempts using additional transgenesis due to low LC expression, we currently are following a strategy of targeted insertion of human LC sequences in the mouse endogenous kappa LC locus. We describe here the first data from this ‘knock-in’ model and propose future prospects to increase the rates of free LC and mimic the human disease. Absence of amyloid deposits in such models would raise the possibility of a resistance to AL amyloidosis in mice and question the feasibility of a reliable animal model for this disease.
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Dates et versions

hal-00617188 , version 1 (26-08-2011)
hal-00617188 , version 2 (12-02-2014)

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Christophe Sirac, Sébastien Bender, Arnaud Jaccard, Franck Bridoux, C. Lacombe, et al.. Strategies to model AL amyloidosis in mice.. Amyloid: The Journal of Protein Folding Disorders, 2011, 18 (Suppl 1), pp.45-47. ⟨10.3109/13506129.2011.574354016⟩. ⟨hal-00617188v2⟩
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