UCHLI in the Mammalian Nervous System
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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.