Fly research helps inform understanding of signal pathway roles in Schimke immuno-osseous dysplasia

Morimoto M, Myung C, Beirnes K, Choi K, Asakura Y, Bokenkamp A, Bonneau D, Brugnara M, Charrow J, Colin E, Davis A, Deschenes G, Gentile M, Giordano M, Gormley AK, Govender R, Joseph M, Keller K, Lerut E, Levtchenko E, Massella L, Mayfield C, Najafian B, Parham D, Spranger J, Stenzel P, Yis U, Yu Z, Zonana J, Hendson G, Boerkoel CF. Increased Wnt and Notch signaling: a clue to the renal disease in Schimke immuno-osseous dysplasia? Orphanet J Rare Dis. 2016 Nov 5;11(1):149. PMID: 27816064; PMCID: PMC5097426.

From the abstract: "Schimke immuno-osseous dysplasia (SIOD) is a multisystemic disorder caused by biallelic mutations in the SWI/SNF-related matrix-associated actin-dependent regulator of chromatin, subfamily A-like 1 (SMARCAL1) gene. ... we hypothesized that SMARCAL1 deficiency causes the focal segmental glomerulosclerosis (FSGS) of SIOD by altering renal gene expression. We tested this hypothesis by gene expression analysis of an SIOD patient kidney and verified these findings through immunofluorescent analysis in additional SIOD patients and a genetic interaction analysis in Drosophila. ... We conclude that increased Wnt and Notch activity result from SMARCAL1 deficiency and, as established causes of FSGS, contribute to the renal disease of most SIOD patients. This further clarifies the pathogenesis of SIOD and will hopefully direct potential therapeutic approaches for SIOD patients."

Review--Drosophila as a model for inflammation, innate immunity and cancer

Wang L, Kounatidis I, Ligoxygakis P. Drosophila as a model to study the role of blood cells in inflammation, innate immunity and cancer. Front Cell Infect Microbiol. 2014 Jan 9;3:113. PMID: 24409421; PMCID: PMC3885817.

Relevant review--fly model & host defense

Ferrandon D. The complementary facets of epithelial host defenses in the genetic model organism Drosophila melanogaster: from resistance to resilience. Curr Opin Immunol. 2013 Feb;25(1):59-70. Review. PMID: 23228366.

Fly model of calcium overload-induced necrosis. Relevance to several diseases. Recent methods report.

Liu K, Li Y, Liu L. Modeling calcium-overload mediated necrosis in Drosophila. Methods Mol Biol. 2013;1004:203-13. PMID: 23733579.

Immunity and age-related disease. Recent review.

This open access review discusses fly models related to aging and immunity.

Eleftherianos I, Castillo JC. Molecular mechanisms of aging and immune system regulation in Drosophila. Int J Mol Sci. 2012;13(8):9826-44. PubMed PMID: 22949833