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Abrogation of both short and long forms of latent transforming growth factor-β binding protein-1 causes defective cardiovascular development and is perinatally lethal.

Matrix Biol.2015 Apr;43:61-70. Epub 2015 Mar 21
Masahito Horiguchi 1 , Vesna Todorovic 2 , Krassimira Hadjiolova 1 , Ralf Weiskirchen 3 , Daniel B Rifkin 4
Masahito Horiguchi 1 , Vesna Todorovic 2 , Krassimira Hadjiolova 1 , Ralf Weiskirchen 3 , Daniel B Rifkin 4

[No authors listed]

Author information
  • 1 Department of Cell Biology, New York University Langone School of Medicine, 550 First Avenue, New York, NY 10016, USA.
  • 2 Department of Cell Biology, New York University Langone School of Medicine, 550 First Avenue, New York, NY 10016, USA; Department of Medicine, New York University Langone School of Medicine, 550 First Avenue, New York, NY 10016, USA.
  • 3 Institute of Molecular Pathobiochemistry, Experimental Gene Therapy and Clinical Chemistry, RWTH University Hospital Aachen, Pauwelsstr. 30, D-52074 Aachen, Germany.
  • 4 Department of Cell Biology, New York University Langone School of Medicine, 550 First Avenue, New York, NY 10016, USA; Department of Medicine, New York University Langone School of Medicine, 550 First Avenue, New York, NY 10016, USA. Electronic address: Daniel.Rifkin@nyumc.org.

摘要


Latent transforming growth factor-β 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-β (TGF-β) 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.

KEYWORDS: Conditional knockout mice, Latent TGF-β binding protein, Persistent truncus arteriosus, Transforming growth factor β