Expressions of immunophilin FKBP-12 and FKBP-52 were examined in the spinal cord of transgenic mice with an ALS-linked mutant Cu/Zn superoxide dismutase (SOD1) gene. The immunoreactivity of FKBP-12 was present predominantly in the cytoplasm, but did not show a difference between age-matched wild type and transgenic (Tg) mice at 25 and 35 weeks. In contrast, the immunoreactivity of FKBP-52 was predominantly present in the nucleus, which progressively declined only in the Tg mice as early as an early presymptomatic stage at 25 weeks of age in the anterior horn neurons. The present result suggests that the downregulation of FKBP-52 may be involved in the pathogenesis in the early stages of amyotrophic lateral sclerosis (ALS).
- Transgenic mouse