Serum Ciliotoxic Activity in Mutant Mice with Some Hereditary Alterations Resembling Cystic Fibrosis

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Abstract
Summary: Serum ciliotoxic activity (SCA), which had been described in cystic fibrosis (CF), was studied in mutant mice with some hereditary alterations resembling this inherited disease. A test was developed to detect SCA in biologic fluids by means of a natural vibratile system; it consisted in the incubation of mouse sperm with different CF serum dilutions under controlled conditions, in order to determine the sperm translation cessation time (STCT) and the sperm activity cessation time (SACT). As the findings of this study were similar to those obtained by other authors who have been employing alternative systems, it was decided to apply this method on mice serum. Sera were obtained from male and female 3- to 4-wk-old mice of DBA/2J-cri, C57BL/ 6J-bgJ, and BALB/c inbred strains; SCA was investigated in affected homozygotes and their +/? siblings were used as controls. Male 8- to 16-wk-old BALB/c mice were employed as sperm donors. Significant differences were found between cri/cri and +/? mice of the DBA/2J-cri strain in both STCT and SACT determinations at 1/10 serum dilution. It is concluded that mouse sperm is a system sensitive enough to detect SCA in biologic fluids from human beings. Likewise, the test of the mouse sperm allowed us to detect SCA in cri mice, turning this mutation into a possible animal model for CF. Speculation: Homozygous mice for the crigene of the DBA/2J-cri strain show electrolyte alterations and a decreased lung clearance ofStaphylococcus aureus The presence of SCA in these mutants, also frequently observed in CF patients, lends support to the concept of a CF animal model.

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