ArticleThe Journal of experimental medicine2017
A p190BRhoGAP mutation and prolonged RhoB activation in fatal systemic capillary leak syndrome.
Article in The Journal of experimental medicine, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
16 citing papers in PubMed, 1 synthesis or guideline pooled it, 26 citations in OpenAlex.
- Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review.European journal of pediatrics · 2018Pooled it
- Non-canonical and constitutive activation of small GTPases: more than an exception to the rule?The Biochemical journal · 2026Review
- Roles of endothelial cells during infection.Current opinion in immunology · 2025Review
- Systemic capillary leak syndrome.Nature reviews. Disease primers · 2024Review
- Hypersensitive blood vessels in Clarkson disease.The Journal of clinical investigation · 2024Article
- Intrinsic endothelial hyperresponsiveness to inflammatory mediators drives acute episodes in models of Clarkson disease.The Journal of clinical investigation · 2024Article
- Chronic systemic capillary leak syndrome with lymphatic capillaries involvement and MYOF mutation: case report and literature review.Frontiers in genetics · 2023Article
- Impaired microtubule dynamics contribute to microthrombocytopenia in RhoB-deficient mice.Blood advances · 2022Article
- ArhGEF12 activates Rap1A and not RhoA in human dermal microvascular endothelial cells to reduce tumor necrosis factor-induced leak.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2022Article
- Tumor necrosis factor-induced ArhGEF10 selectively activates RhoB contributing to human microvascular endothelial cell tight junction disruption.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2021Article
- A preliminary study of influences of hydroxyethyl starch combined with ulinastatin on degree of edema in newborns with capillary leak syndrome.American journal of translational research · 2021Article
- Progenitor-derived human endothelial cells evade alloimmunity by CRISPR/Cas9-mediated complete ablation of MHC expression.JCI insight · 2019Article
- Whole-Exome Sequencing of Adult and Pediatric Cohorts of the Rare Vascular Disorder Systemic Capillary Leak Syndrome.Shock (Augusta, Ga.) · 2019Article
- A natural mouse model reveals genetic determinants of systemic capillary leak syndrome (Clarkson disease).Communications biology · 2019Article
- Rare and Low-Frequency Variant ofCirculation. Genomic and precision medicine · 2018Article
- The Cullin-3-Rbx1-KCTD10 complex controls endothelial barrier function via K63 ubiquitination of RhoB.The Journal of cell biology · 2018Article
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
We describe a fatal case of pediatric systemic capillary leak (Clarkson's disease) associated with a point mutation in p190BRhoGAP. Dermal microvascular endothelial cells (ECs) isolated from this patient form monolayers with similar levels and distribution of junctional proteins and transendothelial electrical resistance compared with normal human dermal microvascular ECs. However, patient-derived ECs demonstrate a greater increase in permeability and impaired recovery of barrier function in response to tumor necrosis factor (TNF) compared with normal donor EC cultures. TNF transiently activates RhoB in ECs coincident with developing leak, and inactivation of RhoB correlates with barrier recovery. The mutation in p190BRhoGAP impairs RhoB inactivation, and the mutant phenotype of patient-derived ECs is replicated by siRNA knockdown of p190BRhoGAP in normal ECs. These data suggest a previously unknown function for p190BRhoGAP in control of capillary EC barrier function that may also be important in acquired systemic capillary leak associated with critical illness in humans.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.