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Functional Coupling between the Extracellular Matrix and Nuclear Lamina by Wnt Signaling in Progeria

  1. Author:
    Hernandez, L.
    Roux, K. J.
    Wong, E. S. M.
    Mounkes, L. C.
    Mutalif, R.
    Navasankari, R.
    Rai, B.
    Cool, S.
    Jeong, J. W.
    Wang, H. H.
    Lee, H. S.
    Kozlov, S.
    Grunert, M.
    Keeble, T.
    Jones, C. M.
    Meta, M. D.,
    Young, S. G.
    Daar, I. O.
    Burke, B.
    Perantoni, A. O.
    Stewart, C. L.

  2. Author Address

    [Hernandez, Lidia; Mounkes, Leslie C.; Wang, Honghe; Kozlov, Serguei; Perantoni, Alan O.; Stewart, Colin L.] NCI, Canc & Dev Biol Lab, Frederick, MD 21702 USA. [Lee, Hyun-Shik; Daar, Ira O.] NCI, Lab Cell & Dev Signaling, Frederick, MD 21702 USA. [Hernandez, Lidia] NCI, Mol Signalling Sect, Med Oncol Branch, Ctr Canc Res, Bethesda, MD 20892 USA. [Roux, Kyle J.; Navasankari, Raju; Burke, Brian] Univ Florida, Coll Med, Dept Anat & Cell Biol, Gainesville, FL 32606 USA. [Wong, Esther Sook Miin; Mutalif, Rafidah; Navasankari, Raju; Rai, Bina; Cool, Simon; Grunert, Martin; Keeble, Thomas; Jones, C. Michael; Stewart, Colin L.] Immunos, Inst Med Biol, Singapore 138648, Singapore. [Jeong, Jae-Wook] Baylor Coll Med, Dept Mol & Cellular Biol, Houston, TX 77030 USA. [Meta, Margarita D.; Young, Stephen G.] Univ Calif Los Angeles, David Geffen Sch Med, Dept Med, Los Angeles, CA 90095 USA.;Stewart, CL, NCI, Canc & Dev Biol Lab, Frederick, MD 21702 USA.;colin.stewart@imb.a-star.edu.sg
    1. Year: 2010
    2. Date: Sep 14
    3. Epub Date: 9/14/2010
  2. Journal: Developmental Cell
    1. 19
    2. 3
    3. Pages: 413-425
  4. Type of Article: Article
  5. ISSN: 1534-5807
  1. Abstract:

    The segmental premature aging disease Hutchinson-Gilford Progeria (HGPS) is caused by a truncated and farnesylated form of Lamin A. In a mouse model for HGPS, a similar Lamin A variant causes the proliferative arrest and death of postnatal, but not embryonic, fibroblasts. Arrest is due to an inability to produce a functional extracellular matrix (ECM), because growth on normal ECM rescues proliferation. The defects are associated with inhibition of canonical Wnt signaling, due to reduced nuclear localization and transcriptional activity of Lef1, but not Tcf4, in both mouse and human progeric cells. Defective Wnt signaling, affecting ECM synthesis, may be critical to the etiology of HGPS because mice exhibit skeletal defects and apoptosis in major blood vessels proximal to the heart. These results establish a functional link between the nuclear envelope/ lamina and the cell surface/ECM and may provide insights into the role of Wnt signaling and the ECM in aging.

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External Sources

  1. View Full Text
  2. DOI: 10.1016/j.devcel.2010.08.013
  3. PMID: 20833363
  4. PMCID: PMC2953243
  5. View record in Web of Science®
  6. WOS: 000282203100010
  7. NIHMSID: Nihms231587

Library Notes

  1. Fiscal Year: FY2009-2010
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