Genetic Diagnosis Of CMT2L Transgenic Mice And Co-localization Analysis Of HSPB8with HSPB1in Sciatic Nerve

Objective It has been confirmed the HSPB8is cauative gene for CMT2L.We intend to carrying the K141N mutation of HSPB8transgenic mice model, Observe the transgenic mouse models of K141NHSPB8ultrastructural Pathology whether HSPB8and HSPB1exist co-localization.Methods pCAGGS-K141NHSPB8passaged transgenic mice by PCR gene identification, take pCAGGS-K141NHSPB8transgenic mice and normal mice sciatic nerve, immunohistochemical double labeling staining after chemical at the electron microscope HSPB8the HSPB1the co-localization of the situation.Results (1) Transgenic mice by gene diagnosis to determine the HSPB8gene423G→T missense mutation.(2) HSPB8protein particles which signed with10nm colloidal gold and HSPB1protein mark20nm colloidal gold expression in PCAGGS-HA-K141NHSPB8mice increased, and the good co-localization between HSPB8and HSPB1.Conclusion (1) Confirmed that the pCAGGS-K141NHSPB8transfer the genetic mouse model was successfully constructed, and a smooth passage to the F7generation.(2) HSPB1may be involved with HSPB8in the pathogenic process of pCAGGS-K141NHSPB8transgenic mice.