Files and links (1)
Published, Version of Record (VoR)CC BY V4.0, Open
Journal article
A natural mouse model reveals genetic determinants of systemic capillary leak syndrome (Clarkson disease)
Communications biology, v 2(1), pp 398-398
2019
PMID: 31701027
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
The systemic capillary leak syndrome (SCLS, Clarkson disease) is a disorder of unknown etiology characterized by recurrent episodes of vascular leakage of proteins and fluids into peripheral tissues, resulting in whole-body edema and hypotensive shock. The pathologic mechanisms and genetic basis for SCLS remain elusive. Here we identify an inbred mouse strain, SJL, which recapitulates cardinal features of SCLS, including susceptibility to histamine- and infection-triggered vascular leak. We named this trait "Histamine hypersensitivity" (Hhs/
) and mapped it to Chromosome 6.
is syntenic to the genomic locus most strongly associated with SCLS in humans (3p25.3), revealing that the predisposition to develop vascular hyperpermeability has a strong genetic component conserved between humans and mice and providing a naturally occurring animal model for SCLS. Genetic analysis of
may reveal orthologous candidate genes that contribute not only to SCLS, but also to normal and dysregulated mechanisms underlying vascular barrier function more generally.
Metrics
Details
- Title
- A natural mouse model reveals genetic determinants of systemic capillary leak syndrome (Clarkson disease)
- Creators
- Abbas Raza - University of VermontZhihui Xie - National Institute of Allergy and Infectious DiseasesEunice C Chan - National Institute of Allergy and Infectious DiseasesWei-Sheng Chen - National Institute of Allergy and Infectious DiseasesLinda M Scott - National Institute of Allergy and Infectious DiseasesA Robin Eisch - Lung and Vascular Inflammation Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD 20892 USADimitry N Krementsov - University of VermontHelene F Rosenberg - National Institute of Allergy and Infectious DiseasesSamir M Parikh - Beth Israel Deaconess Medical CenterElizabeth P Blankenhorn - Drexel UniversityCory Teuscher - University of VermontKirk M Druey - National Institute of Allergy and Infectious Diseases
- Publication Details
- Communications biology, v 2(1), pp 398-398
- Publisher
- Springer Nature
- Grant note
- Z01 AI000943 / Intramural NIH HHS Z01 AI000746 / Intramural NIH HHS
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Microbiology and Immunology
- Web of Science ID
- WOS:000493556700002
- Scopus ID
- 2-s2.0-85074259575
- Other Identifier
- 991019168754504721
UN Sustainable Development Goals (SDGs)
This publication has contributed to the advancement of the following goals:
InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Biology