Ubiquitin Carboxyl-terminal Hydrolase Isozyme L1 (UCH-L1, UCHL1, Neuron Cytoplasmic Protein 9.5, PGP 9.5, PGP9.5, Ubiquitin Thioesterase L1)

Ubiquitin C-terminal hydrolase 1 (UCHL1) has several other names, such 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 (1). The protein is extremely abundant, and was estimated to be present at a concentration of 200-500 micrograms/g wet weight, representing a major protein component of neuronal cytoplasm (1). This has been claimed to represent 1-2% of total brain protein. It was later found that a ubiquitin C-terminal hydrolase enzyme activity was associated with the PGP9.5 protein, resulting in the renaming of PGP9.5 to ubiquitin C-terminal hydrolase 1. This is the first of a family of ubiquitin C-terminal hydrolases which have been characterized, and is expressed heavily in neurons in the brain. The ubiquitin C-terminal hydrolases cleave ubiquitin from other molecules. This activity is important to generate mono-ubiquitin from genes which encode polyubiquitin chains or ubiquitin fused to other proteins. The activity is also important to remove ubiquitin from partially degraded proteins, allowing the ubiquitin monomer to be recycled. Regulation of the ubiquitin pathway is very important and many disease states are associated with defects in this pathway. The covalent ubiquitin conjugates may then be degraded in the proteasome. Point mutations in the UCHL1 gene are associated with some forms of human Parkinson's disease (3). Recent studies suggest that UCHL1 also has a ubiqutinyl ligase activity, being able to couple ubiquitin monomers by linking the C-terminus of one with lysine 63 of the other (3). Since UCHL1 is heavily expressed in neurons, antibodies to UCHL1 can be used to identify neurons in histological sections and in tissue culture. The great abundance of this protein in neurons means that it is released from neurons in large amounts following injury or degeneration, so the detection of of UCHL1 in CSF and other bodily fluids can be used as a biomarker. UCHL1 was also discovered as a gene mutated in some rare famial forms of Parkinson's disease. Park5 was characterized as the gene causative of this form of Parkinson's and on analysis the Park5 gene proved to encode an I93M point mutations in the UCHL1 gene, which reduces the ubiquitin hydrolase activity. Interestingly a common allelic variant of UCHL1, the S18Y polymorphism is actually protective against Parkinson's disease. For a recent excellent review co-authored by the discoverer of UCHL1/Pgp9.5 (4). The HGNC name for this protein is UCHL1.\n\nApplications:\nSuitable for use in Immunofluorescence and Western Blot. A suitable control tissue is rat spinal cord or peripheral nerve homogenate. The UCHL1 protein runs at about 27kD on SDS-PAGE gels, and is a prominent component of brain, spinal cord and especially cortical extracts. Other applications not tested.\n\nRecommended Dilution:\nImmunofluorescence: 1:1000\nWestern Blot: 1:10,000\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

031439

Size

100ul

Applications

IF, WB

Hosts

Ch/Bird

Reactivities

Hum, Rat

Form

10mM sodium azide

P Type

Pab

Purity

Purified

Isotype

IgY

References

1. 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).\n\n2. 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).\n\n3. Liu Y, Fallon L, Lashuel HA, Liu Z, Lansbury PT Jr. The UCH-L1 gene encodes two opposing enzymatic activities that affect alpha-synuclein degradation and Parkinson's disease susceptibility. Cell 111:209-18 (2002).\n\n4. Day IN, Thompson RJ. UCHL1 (PGP 9.5): Neuronal biomarker and ubiquitin system protein. Prog Neurobiol. 2009 Oct 30.

Additional Info

Recognizes UCHL1 in human and rat.

Alternative Names

Ubiquitin C-terminal Hydrolase 1, EC=3.4.19.12, EC=6.-.-.-