| First Author | Horiguchi M | Year | 2015 |
| Journal | Matrix Biol | Volume | 43 |
| Pages | 61-70 | PubMed ID | 25805620 |
| Mgi Jnum | J:224402 | Mgi Id | MGI:5662169 |
| Doi | 10.1016/j.matbio.2015.03.006 | Citation | Horiguchi M, et al. (2015) Abrogation of both short and long forms of latent transforming growth factor-beta binding protein-1 causes defective cardiovascular development and is perinatally lethal. Matrix Biol 43:61-70 |
| abstractText | Latent transforming growth factor-beta binding protein-1 (LTBP-1) is an extracellular protein that is structurally similar to fibrillin and has an important role in controlling transforming growth factor-beta (TGF-beta) signaling by storing the cytokine in the extracellular matrix and by being involved in the conversion of the latent growth factor to its active form. LTBP-1 is found as both short (LTBP-1S) and long (LTBP-1L) forms, which are derived through the use of separate promoters. There is controversy regarding the importance of LTBP-1L, as Ltbp1L knockout mice showed multiple cardiovascular defects but the complete null mice did not. Here, we describe a third line of Ltbp1 knockout mice generated utilizing a conditional knockout strategy that ablated expression of both L and S forms of LTBP-1. These mice show severe developmental cardiovascular abnormalities and die perinatally; thus these animals display a phenotype similar to previously reported Ltbp1L knockout mice. We reinvestigated the other "complete" knockout line and found that these mice express a splice variant of LTBP-1L and, therefore, are not complete Ltbp1 knockouts. Our results clarify the phenotypes of Ltbp1 null mice and re-emphasize the importance of LTBP-1 in vivo. |
