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Mouse models of amyloidosis

Amyloidosis is a rare disease caused by extracellular deposition of insoluble abnormal fibrils derived from aggregation of a misfolded variant of a normally soluble protein. Apolipoprotein (apo) AII is a major protein of HDL (high density lipoproteins) synthesized in liver. An apo AII variant carrying a mutation in the Stop codon causes an autosomal dominant apoa2-amyloidosis in humans (Yazaki et al, Kidney Int 2001, 60:1658-65). As in humans, our transgenic mice for the mutant human apo a2 gene with a Stop codon to Serine mutation (Stop78->Ser) express a longer hapo AII protein (99 AA instead of 77). Characteristics of transgenic mice:
• Spontaneous systemic amyloidosis begins as early as 3-4 months of age and increases as a function of age, with no need for an inflammatory state. Mouse lifespan varies between 6 and 9 months, depending on the gravity of amyloidosis.
• Amyloid fibrils stain with Congo red with the characteristic green birefringence under polarized light.
• Amyloid fibrils have been characterized by immunohistochemistry with human-apoAII antibodies in kidney, liver, heart spleen, and by electron microscopy in capillaries of liver and heart.
• Amyloid fibrils have been isolated from tissues; solubilized amyloid fibril protein was fractionated and the amino acid sequence of full-length hapoAII with a 21 amino acid carboxyl terminal extension was verified.

Interest / Relevance: • This is the first animal model of hereditary systemic amyloidosis with a rapid and massive development, without need to infect the mice.
• It is a new tool for testing in vivo and ex-vivo detection techniques and therapeutic strategies targeting all systemic amyloidoses.
• Despite amyloidosis, mice are fertile.
Publications:
Transient micro-elastography: a novel non-invasive approach to measure liver stiffness in mice. Bastard C.Bosisio MR. Chabert M. Kalopissis AD. Mahrouf-Yorgov M. Gilgenkrantz H. Mueller S. Sandrin L.; World J Gastroenterol. World J Gastroenterol 2011 February 28; 17(8): 968-975 wjg@wjgnet.com doi:10.3748/wjg.v17.i8.968

Reference:

RT00462

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Athina-Despina KALOPISSIS
Inserm Transfert
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Species: Mouse
Genotype: Hemizygous for a mutated human apoA2 gene, under the control of the homologous promoter (-911/+2045).
Strain: B6.Tg(hApoA2 Stop78->Ser)
Genetic Background: C57BL/6
Applications: Models available : Three transgenic lines are available: ? Line F, characterized by early-onset (3-4 months) systemic amyloidosi
Rare disease:
Last update: 14/01/2022

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