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Cited by 256

The DOI matches its Crossref record; 33 of the 33 references it cites were found in a registry or an index.

Disruption of the gene encoding the latent transforming growth factor-β binding protein 4 (LTBP-4) causes abnormal lung development, cardiomyopathy, and colorectal cancer

Open access
Sterner-Kock, Anja, Thorey, Irmgard S., Koli, Katri, Wempe, Frank, Otte, Jürgen, Bangsow, Thorsten, Kuhlmeier, Katharina, Kirchner, Thomas, Jin, Shenchu, Keski-Oja, Jorma, von Melchner, Harald·2002·Genes & Development
DOI 10.1101/gad.229102
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Abstract

Transforming growth factor-βs (TGF-βs) are multifunctional growth factors that are secreted as inactive (latent) precursors in large protein complexes. These complexes include the latency-associated propeptide (LAP) and a latent transforming growth factor-β binding protein (LTBP). Four isoforms of LTBPs (LTBP-1–LTBP-4) have been cloned and are believed to be structural components of connective tissue microfibrils and local regulators of TGF-β tissue deposition and signaling. By using a gene trap strategy that selects for integrations into genes induced transiently during early mouse development, we have disrupted the mouse homolog of the human LTBP-4 gene. Mice homozygous for the disrupted allele develop severe pulmonary emphysema, cardiomyopathy, and colorectal cancer. These highly tissue-specific abnormalities are associated with profound defects in the elastic fiber structure and with a reduced deposition of TGF-β in the extracellular space. As a consequence, epithelial cells have reduced levels of phosphorylated Smad2 proteins, overexpress c-myc, and undergo uncontrolled proliferation. This phenotype supports the predicted dual role of LTBP-4 as a structural component of the extracellular matrix and as a local regulator of TGF-β tissue deposition and signaling.

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Metadata

Authors
Sterner-Kock, A., Thorey, I. S., Koli, K., Wempe, F., Otte, J., Bangsow, T., Kuhlmeier, K., Kirchner, T., Jin, S., Keski-Oja, J., & von Melchner, H.
Venue
Genes & Development
Publisher
Cold Spring Harbor Laboratory
Published
2002-09-01
Volume
16
Issue
17
Pages
2264-2273
ISSN
  • 0890-9369 (ISSN-L)
  • 1549-5477
DOI
10.1101/gad.229102
OpenAlex
W2139930644
Cited by
256
References
33
Type
posted content
Language
en
Open access
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CoChat. (2026). Citation check: Disruption of the gene encoding the latent transforming growth factor-β binding protein 4 (LTBP-4) causes abnormal lung development, cardiomyopathy, and colorectal cancer. https://app.cochat.ai/papers/10.1101/gad.229102
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Sterner-Kock, A., Thorey, I. S., Koli, K., Wempe, F., Otte, J., Bangsow, T., Kuhlmeier, K., Kirchner, T., Jin, S., Keski-Oja, J., & von Melchner, H. (2002). Disruption of the gene encoding the latent transforming growth factor-β binding protein 4 (LTBP-4) causes abnormal lung development, cardiomyopathy, and colorectal cancer. Genes & Development. https://doi.org/10.1101/gad.229102

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.