Pkd1 V: Uncleavable Pkd1 hypomorhic allele

Description: This knock-in mouse model has an amino acid substitution at position T3041V of the G-protein coupled receptor proteolytic site (GPS) of polycystin 1, disrupting normal cleavage. Heterozygous mice are phenotypically normal. Homozygotes are phenotypically normal at birth but develop cysts and die within 2-3 weeks.

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