This strain is a Ttr humanized model and can be used for research on ATTRv-PN. A human TTR gene fragment carrying the V50M mutation, driven by the mouse Alb promoter (liver-specific promoter), is inserted at the H11 safe harbor. This modification does not affect the expression of the mouse Ttr gene. The homozygous H11-Alb-hTTR*V50M mice are viable and fertile.