{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T13:29:34Z","timestamp":1792589374697,"version":"4.5.0"},"reference-count":52,"publisher":"National Academy of Sciences","issue":"4","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2010,1,26]]},"abstract":"<jats:p>\n                    The enzyme ubiquitin carboxyl-terminal hydrolase L1 (UCH-L1) is one of the most abundant proteins in the mammalian nervous system. In humans, UCH-L1 is also found in the ubiquitinated inclusion bodies that characterize neurodegenerative diseases in the brain, suggesting its involvement in neurodegeneration. The physiologic role of UCH-L1 in neurons, however, remains to be further elucidated. For example, previous studies have provided evidence both for and against the role of UCH-L1 in synaptic function in the brain. Here, we have characterized a line of knockout mice deficient in the\n                    <jats:italic>UCH-L1<\/jats:italic>\n                    gene. We found that, in the absence of UCH-L1, synaptic transmission at the neuromuscular junctions (NMJs) is markedly impaired. Both spontaneous and evoked synaptic activity are reduced; paired pulse-facilitation is impaired, and synaptic transmission fails to respond to high-frequency, repetitive stimulation at the NMJs of\n                    <jats:italic>UCH-L1<\/jats:italic>\n                    knockout mice. Morphologic analyses of the NMJs further revealed profound structural defects\u2014loss of synaptic vesicles and accumulation of tubulovesicular structures at the presynaptic nerve terminals, and denervation of the muscles in\n                    <jats:italic>UCH-L1<\/jats:italic>\n                    knockout mice. These findings demonstrate that UCH-L1 is required for the maintenance of the structure and function of the NMJ and that the loss of normal UCH-L1 activity may result in neurodegeneration in the peripheral nervous system.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0911516107","type":"journal-article","created":{"date-parts":[[2010,1,4]],"date-time":"2010-01-04T21:36:36Z","timestamp":1262640996000},"page":"1636-1641","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":129,"title":["Ubiquitin carboxyl-terminal hydrolase L1 is required for maintaining the structure and function of the neuromuscular junction"],"prefix":"10.1073","volume":"107","author":[{"given":"Fujun","family":"Chen","sequence":"first","affiliation":[{"name":"Department of Neuroscience, University of Texas Southwestern Medical Center, Dallas, TX 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