Ubiquitin Hydrolase 1 Antibody [MCA-BH7]

Ubiquitin C-terminal hydrolase 1 (UCHL1) is also known as ubiquitin carboxyl esterase L1, ubiquitin thiolesterase, neuron-specific protein PGP9.5 and Park5. It was originally identified as a major component of the neuronal cytoplasm from 2-dimensional gel analysis of brain tissues, and was given the name PGP9.5. It was later found that ubiquitin C-terminal hydrolase enzyme activity was associated with the PGP9.5 protein. The ubiquitin C-terminal hydrolases cleave ubiquitin from other molecules. Regulation of the ubiquitin pathway is very important and many disease states are associated with defects in this pathway. Genetic knockout of UCHL1 in mice results in a motor neuron degeneration similar to the spontaneous gracile axonal dystrophy (gad) mutant mice. Point mutations in the UCHL1 gene are associated with some forms of human Parkinson's disease. Since UCHL1 is heavily expressed in neurons, it is released in large amounts following injury or degeneration, so the detection of UCHL1 in CSF and other bodily fluids can be used as a biomarker.

Doran JF, Jackson P, Kynoch PA, Thompson RJ. Isolation of PGP 9.5, a new human neurone-specific protein detected by high-resolution two-dimensional electrophoresis. J Neurochem. 40:1542-7 (1983) .

Wilkinson KD, Lee KM, Deshpande S, Duerksen-Hughes P, Boss JM, Pohl J. The neuron-specific protein PGP 9.5 is a ubiquitin carboxyl-terminal hydrolase. Science. 1989 246:670-3 (1989) .

Kurihara LJ, Kikuchi T, Wada K, Tilghman SM. Loss of Uch-L1 and Uch-L3 leads to neurodegeneration, posterior paralysis and dysphagia. Hum Mol Genet. 10:1963-70 (2001) .