| dc.description |
UCHL1 , or Ubiquitin Carboxy Terminal Hydrolase L1 , is a de -ubiquitinating enzyme (DUB ) thought to be involved in ubiquitin recycling . Beyond this , its natural function is almost wholly unknown . Mice lacking this enzyme exhibit a severe and rapidly progressing neurodegenerative phenotype . Using one of these mouse models , in which a lacz -neo construct is cloned in under the UCHL1 promoter , we have studied the gross progression of phenotype of these mice , characterized the expression pattern of UCHLO1 in brain , spinal cord , and lumbar dorsal root ganglia , described degeneration of muscle spindles , and established a timecourse of degeneration of neuromuscular junctions . Presented in this thesis is the culmination of these studies . |
|