Multi‐organ phenotypes in mice lacking latent TGFβ binding protein 2 (LTBP2)

Background Latent TGFβ binding protein‐2 (LTBP2) is a fibrillin 1 binding component of the microfibril. LTBP2 is the only LTBP protein that does not bind any isoforms of TGFβ, although it may interfere with the function of other LTBPs or interact with other signaling pathways. Results Here, we inves...

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Bibliographic Details
Published in:Developmental dynamics 2024-02, Vol.253 (2), p.233-254
Main Authors: Bodmer, Nicholas K., Knutsen, Russell H., Roth, Robyn A., Castile, Ryan M., Brodt, Michael D., Gierasch, Carrie M., Broekelmann, Thomas J., Gibson, Mark A., Haspel, Jeffrey A., Lake, Spencer P., Koenitzer, Jeffrey R., Brody, Steven L., Silva, Matthew J., Mecham, Robert P., Ornitz, David M.
Format: Article
Language:English
Subjects:
Animals
Aorta
Body fat
Bone mass
Carrier Proteins - genetics
Carrier Proteins - metabolism
Extracellular matrix
Extracellular Matrix - metabolism
Fibrillin
fibroblasts
Isoforms
LTBP2
LTBPs
Mice
myofibroblasts
Phenotype
Phenotypes
Protein Binding
Protein Isoforms - metabolism
Proteins
Signal transduction
TGFβ
Transforming Growth Factor beta - metabolism
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Summary:Background Latent TGFβ binding protein‐2 (LTBP2) is a fibrillin 1 binding component of the microfibril. LTBP2 is the only LTBP protein that does not bind any isoforms of TGFβ, although it may interfere with the function of other LTBPs or interact with other signaling pathways. Results Here, we investigate mice lacking Ltbp2 (Ltbp2−/−) and identify multiple phenotypes that impact bodyweight and fat mass, and affect bone and skin development. The alterations in skin and bone development are particularly noteworthy since the strength of these tissues is differentially affected by loss of Ltbp2. Interestingly, some tissues that express high levels of Ltbp2, such as the aorta and lung, do not have a developmental or homeostatic phenotype. Conclusions Analysis of these mice show that LTBP2 has complex effects on development through direct effects on the extracellular matrix (ECM) or on signaling pathways that are known to regulate the ECM. Key Findings Ltbp2 deficiency results in trabecular bone overgrowth Ltbp2 deficiency results in skin alterations Ltbp2 deficiency may impact metabolism Ltbp2 deficiency does not result in obvious alterations of lung and aortal tissue
ISSN:1058-8388
1097-0177
DOI:10.1002/dvdy.651