The dark sides of capillary morphogenesis gene 2

Ecole Polytechnique Fédérale de Lausanne, Institute of Global Health, Lausanne, Switzerland.
The EMBO Journal (Impact Factor: 10.43). 11/2011; 31(1):3-13. DOI: 10.1038/emboj.2011.442
Source: PubMed


Capillary morphogenesis gene 2 (CMG2) is a type I membrane protein involved in the homeostasis of the extracellular matrix. While it shares interesting similarities with integrins, its exact molecular role is unknown. The interest and knowledge about CMG2 largely stems from the fact that it is involved in two diseases, one infectious and one genetic. CMG2 is the main receptor of the anthrax toxin, and knocking out this gene in mice renders them insensitive to infection with Bacillus anthracis spores. On the other hand, mutations in CMG2 lead to a rare but severe autosomal recessive disorder in humans called Hyaline Fibromatosis Syndrome (HFS). We will here review what is known about the structure of CMG2 and its ability to mediate anthrax toxin entry into cell. We will then describe the limited knowledge available concerning the physiological role of CMG2. Finally, we will describe HFS and the consequences of HFS-associated mutations in CMG2 at the molecular and cellular level.

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Available from: Francoise Gisou van der Goot
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    • "Over the past 14 years, intensive research efforts have been spent to investigate the two receptors and a great deal of knowledge has been generated. Anthrax toxin and anthrax toxin receptors have been comprehensively reviewed in several excellent review articles567891011. Here, we provide an updated review of the most recent research progress concerning ANTXR2, the major receptor for anthrax toxin in vivo, in anthrax toxin action. "

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    • "Furthermore, TEM8 and CMG2 receptors play a role in epithelial and endothelial cell functions, so that mutations of TEM8 and CMG2 lead to very rare diseases, whose pathological mechanism is still largely unknown (Deuquet et al., 2011). TEM8 is involved in the regulation of expression of vascular endothelial growth factor receptors (VEGFRs), playing a role in angiogenesis that, in turn, is detrimental in cancer progression (Deuquet et al., 2011). CMG2 is involved in the regulation cytoskeleton structure and might have a role in cancer spreading (Cryan and Rogers, 2011). "
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