What does this research mean for the field?

Expression of the NRAS Q61R mutation in lymphatic endothelial cells induces a phenotype in mice that closely resembles human Kaposiform Lymphangiomatosis (KLA), establishing a viable in vivo model for the disease. Novelty: ClaimNovelty.METHODOLOGICAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

The study aims to investigate the impact of NRAS Q61R mutation in lymphatic endothelial cells on vessel formation and mortality.

April 19, 2026Pediatric Blood & Cancer1 citations

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NRAS Q61R Expression in Lymphatic Endothelial Cells Causes Enlarged Vessels, Hemorrhagic Chylous Effusions, and High Mortality in a Mouse Model of Kaposiform Lymphangiomatosis

CMC. Griffin McDaniel PPPatricia Pastura JSJoshua P. Scallan

Key Points

The study aims to investigate the impact of NRAS Q61R mutation in lymphatic endothelial cells on vessel formation and mortality.
Used a mouse model with NRAS Q61R mutation
Monitored for chylothorax and vessel enlargement
Assessed Ang-2 levels and mortality rates
Mice with NRAS Q61R exhibited enlarged lymphatic vessels
High mortality rates aligned with KLA patients
Elevated Ang-2 levels were detected in affected mice

Abstract

NRASQ61R mutant mice closely resemble KLA patients with high mortality, hemorrhagic chylothorax, elevated Ang-2 levels, and enlarged lymphatic vessels. Future studies can utilize this model to elucidate the mechanisms underlying KLA pathogenesis and test novel therapeutics.

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NRAS Q61R Expression in Lymphatic Endothelial Cells Causes Enlarged Vessels, Hemorrhagic Chylous Effusions, and High Mortality in a Mouse Model of Kaposiform Lymphangiomatosis

Key Points

Abstract

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