Dataset Information
The STRIPAK complex components FAM40A and FAM40B regulate endothelial cell contractility via ROCKs.
Ontology highlight
ABSTRACT: Background
Endothelial cells provide a barrier between blood and tissues, which is regulated to allow molecules and cells in out of tissues. Patients with cerebral cavernous malformations (CCM) have dilated leaky blood vessels, especially in the central nervous system. A subset of these patients has loss-of-function mutations in CCM3. CCM3 is part of the STRIPAK protein complex that includes the little-characterized proteins FAM40A and FAM40B.Results
We show here that FAM40A and FAM40B can interact with CCM3. Knockdown of CCM3, FAM40A or FAM40B in endothelial cells by RNAi causes an increase in stress fibers and a reduction in loop formation in an in vitro angiogenesis assay, which can be reverted by inhibiting the Rho-regulated ROCK kinases. FAM40B depletion also increases endothelial permeability.Conclusions
These results demonstrate the importance of the FAM40 proteins for endothelial cell physiology, and suggest that they act as part of the CCM3-containing STRIPAK complex.
SUBMITTER: Suryavanshi N
PROVIDER: S-EPMC6276190 | biostudies-literature | 2018 Dec
REPOSITORIES: biostudies-literature
Publications
The STRIPAK complex components FAM40A and FAM40B regulate endothelial cell contractility via ROCKs.
BMC cell biology 20181203 1
<h4>Background</h4>Endothelial cells provide a barrier between blood and tissues, which is regulated to allow molecules and cells in out of tissues. Patients with cerebral cavernous malformations (CCM) have dilated leaky blood vessels, especially in the central nervous system. A subset of these patients has loss-of-function mutations in CCM3. CCM3 is part of the STRIPAK protein complex that includes the little-characterized proteins FAM40A and FAM40B.<h4>Results</h4>We show here that FAM40A and ...[more]