The dark sides of capillary morphogenesis gene 2.

Review 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). The group of Gisou van der Goot ( Van der Goot Lab VDG) here review what is known about the structure of CMG2 and its ability to mediate anthrax toxin entry into cell and describe the limited knowledge available concerning the physiological role of CMG2. Finally, they describe HFS and the consequences of HFS-associated mutations in CMG2 at the molecular and cellular level.