Protein Gene Product 9.5 (PGP9.5,PGP-9.5,Gracile Axonal Dystrophy,Neuron Cytoplasmic Protein 9.5,Parkinson Disease 5,Park5,Ubiquitin Carboxyl Terminal Esterase L1,Ubiquitin C-terminal Esterase L1,Ubiquitin Carboxyl Termi

Antibodies / Antibodies-Ubiquitin

-20°C

Supplied as a liquid in 10mM sodium azide.

Recombinant full length human UCHL1 purified from E. coli.

Ubiquitin C-terminal hydrolase 1 (UCHL1) 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. The protein is extremely abundant representing 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. Point mutations in the UCHL1 gene are associated with some forms of human Parkinson's disease. 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. 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. Interestingly a common allelic variant of UCHL1, the S18Y polymorphism is actually protective against Parkinson's disease.

Applications:
Suitable for Immunofluorescence and Western Blot. Other Applications not tested.

Recommended Dilution:
Immunofluorescence: 1:1000
Western Blot: 1:10000
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.