Article
LTBP1 promotes fibrillin incorporation into the extracellular matrix
2021-12-17
Abstract excerpt
LTBP1 is a large extracellular matrix protein and an associated ligand of fibrillinmicrofibrils. Knowledge of LTBP1 functions is largely limited to its role in targeting and sequestering TGFβ growth factors within the extracellular matrix, thereby regulating their bioavailability. However, the recent description of a wide spectrum of phenotypes in multiple tissues in patients harboring LTBP1 pathogenic variants su...
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Identifiers and source
- Literature Corpus work
- c970ffba-1d3d-58f7-adc2-4d1fb20b871a
- DOI
- 10.1101/2021.12.16.473056
