Mice

RPT3^F/F mice on C57/Bl6 background were obtained from The Center for Animal Resources and Development (CARD), Kumamoto University, Japan²⁰ and crossed with the Sftpc^CreERT2 mice [on a mixed C57:129 genetic background⁴⁹]. Genotyping was performed from tail biopsies; RPT3^F/F mice were genotyped with allele specific primers as described previously by Tashiro et al.²⁰ and primers recommended by the Jackson Laboratory were used to distinguish between heterozygosity and homozygosity for the Cre allele. 8–12-week old female and male Sftpc^WT/CreER:RPT3^F/F and Sftpc^WT/CreER control mice were provided ad libitum access to tamoxifen chow (400 mg tamoxifen citrate, Envigo). Mice were housed in a pathogen-free barrier facility. All procedures were performed under protocols (IACUC2015-0073: TEW) approved by the Institutional Animal Care and Use Committee of Cincinnati Children’s Hospital Medical Center in accordance with National Institutes of Health guidelines. Mice were euthanized when they demonstrated any of the following symptoms: kyphosis, severe lethargy and inactivity as observed by a lack of response when gently prodded, respiratory distress at rest as indicated by deep abdominal excursions, or weight loss of ≥20%.