Expression of peripherin in ubiquinated inclusions of amyotrophic lateral sclerosis

摘要: We evaluated the expression of type III intermediate filament (IF) protein, peripherin (PRP), in ubiquinated inclusions motor neurons amyotrophic lateral sclerosis (ALS). A previous study showed that overexpression PRP transgenic mice induces neuron disease with formation PRP-containing before onset symptoms [J. Cell Biol. 147 (3) (1999) 531]. To determine whether occur human disease, we applied doublelabeling immunofluorescence to paraffin sections spinal cord obtained by autopsy 40 ALS patients sporadic and 39 controls. Inclusions expressed immunoreactive ubiquitin were recorded video camera, stained hematoxylin eosin (HE all LBLIs peripherin. Skein-like (SLIs) identified ubiquitin, but did not express rare exceptions. Neither skein-like nor αB-crystallin, neurofilament protein (NF-L, NF-M NF-H subunits), α-internexin, actin or α-synuclein. Immunoblot whole exhibited a single 57-kDa band Our data document LBLIs, which may provide clue pathogenesis neurodegeneration ALS.