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IFRD1 Gene Linked to Severity of Cystic Fibrosis

Protein appears to influence airway disease by regulating neutrophil effector function

MONDAY, Mar. 2 (HealthDay News) -- The IFRD1 gene appears to play a role in the severity of cystic fibrosis lung disease through its influence on neutrophil effector function, according to research published online Feb. 25 in the journal Nature.

YuanYuan Gu, M.D., of the Cincinnati Children's Hospital Research Foundation in Ohio, and colleagues focused on IFRD1 after analyzing data from a genome-wide single nucleotide polymorphism scan and validation in two cohorts of individuals with the disease and family members.

Using Ifrd1-/- mice, the investigators found that IFRD1 has an important role in controlling neutrophil effector function. Further experimentation found that when these mice were infected with P. aeruginosa, bacterial clearance was slowed, but the mice had less disease and less airway inflammation, the report indicates.

"Despite considerable progress in cystic fibrosis therapy over recent decades, the norm is still an inexorable decline in pulmonary function. Identification of genes modifying cystic fibrosis lung disease, and delineation of the pathogenetic pathways that they influence, holds promise for the development of novel therapeutic strategies. The current data suggest likely utility for therapeutic targeting of neutrophils in this devastating disease. These data also suggest that the IFRD1/HDAC axis may provide a tractable therapeutic target in cystic fibrosis, and other diseases in which neutrophils play an important pathogenetic role," the authors conclude.

Two co-authors disclosed financial relationships with JK Autoimmunity Inc.

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