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

Q: 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.

C. Griffin McDaniel; Patricia Pastura; Joshua P. Scallan; Timothy D. Le Cras

doi:10.1002/1545-5017.70339

April 16, 2026Pediatric Blood & Cancer

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

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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

Implication

Mouse model reveals NRAS Q61R drives hemorrhagic chylothorax and high mortality in Kaposiform Lymphangiomatosis, suggesting new research directions.

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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Cite This Study

McDaniel et al. (2026) studied this question.

synapsesocial.com/papers/69e473de010ef96374d8f90f https://doi.org/10.1002/1545-5017.70339

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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

Implication

Abstract

Discussion

Cite This Study

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

Implication

Abstract

Discussion

Cite This Study

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