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LTBP1 is a large extracellular matrix protein and an associated ligand of fibrillin-microfibrils. 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 suggests a multifaceted role of the protein in the homeostasis of connective tissues. To better understand the human pathology caused by LTBP1 deficiency it is important to investigate its functional role in extracellular matrix formation. In this study, we show that LTBP1 coordinates the incorporation of fibrillin-1 and -2 into the extracellular matrix in vitro. We also demonstrate that this function is differentially exerted by the two isoforms, the short and long forms of LTBP1. Thereby our findings uncover a novel TGFβ-independent LTBP1 function potentially contributing to the development of connective tissue disorders. Copyright © 2022 Elsevier B.V. All rights reserved.


Matthias Przyklenk, Veronika S Georgieva, Fabian Metzen, Sebastian Mostert, Birgit Kobbe, Bert Callewaert, Gerhard Sengle, Bent Brachvogel, Robert P Mecham, Mats Paulsson, Raimund Wagener, Manuel Koch, Alvise Schiavinato. LTBP1 promotes fibrillin incorporation into the extracellular matrix. Matrix biology : journal of the International Society for Matrix Biology. 2022 Jun;110:60-75

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PMID: 35452817

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