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Article

A Griscelli syndrome type 2 murine model of hemophagocytic lymphohistiocytosis (HLH).

European journal of immunology - 1 Nov 2008

Pachlopnik Schmid Jana, Ho Chen-Hsuan, Diana Julien, Pivert Gérard, Lehuen Agnès, Geissmann Frédéric, Fischer Alain, de Saint Basile Geneviève

Abstract excerpt

Griscelli syndrome type 2 is caused by mutations in the RAB27A gene and is a rare and potentially fatal immune disorder associated with hemophagocytic lymphohistiocytosis (HLH). Animal models could provide assistance for better understanding the mechanisms and finding new treatments. Rab27a-deficient (ashen) mice do not spontaneously develop HLH. When injected with lymphocytic choriomeningitis virus (LCMV) strain...

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Source

Identifiers

PMID: 18991284
DOI: 10.1002/eji.200838488

Genes

CCL5
TNF

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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