Article
TGFβ-2 haploinsufficiency causes early death in mice with Marfan syndrome.
Matrix biology : journal of the International Society for Matrix Biology - 1 Aug 2023
Sachan Nalani, Phoon Colin K L, Zilberberg Lior, Kugler Matthias C, Ene Taylor, Mintz Shana B, Murtada Sae-Il, Weiss Dar, Fishman Glenn I, Humphrey Jay D, Rifkin Daniel B
Abstract excerpt
To assess the contribution of individual TGF-β isoforms to aortopathy in Marfan syndrome (MFS), we quantified the survival and phenotypes of mice with a combined fibrillin1 (the gene defective in MFS) hypomorphic mutation and a TGF-β1, 2, or 3 heterozygous null mutation. The loss of TGF-β2, and only TGF-β2, resulted in 80% of the double mutant animals dying earlier, by postnatal day 20, than MFS only mice. Death...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.