Mouse UCHL1 Monoclonal Antibody | anti-UCHL1 antibody

UCHL1 Antibody

Cat. Num. AAA415209

• Gene Names: UCHL1; PARK5; PGP95; PGP9.5; Uch-L1; PGP 9.5
• Applications: Immunofluorescence, Western Blot
• Purity: Affinity purified
• Synonyms: UCHL1; Monoclonal Antibody; UCHL1 Antibody; anti-UCHL1 antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG1
• Purity/Purification: Affinity purified
• Form/Format: Liquid
• Concentration: 100ug/100ul (varies by lot)
• Sequence Length: 223

• Applicable Applications for anti-UCHL1 antibody: Immunofluorescence (IF), Western Blot (WB)
• Application Notes: Try at dilutions of 1:2,000 for immunofluorescence. For Western blots try at 1:20,000.
• Immunogen: 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 (2). 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 UCHL1. This is the first of a family of ubiquitin C-terminal hydrolases which have been characterized, many of which also have rigid cell type specific expression patterns. The ubiquitin C-terminal hydrolases cleave ubiquitin from other molecules. This activity is important to generate mono-ubiquitin from the several 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. For example the Park5 gene causes one form of human Parkinson's disease, and proves to be a point mutations in the UCHL1 gene producing a I93M form of the UCHL1 protein which has reduced ubiquitin hydrolase activity (3). Interestingly a common allelic variant of UCHL1, the S18Y polymorphism is actually protective against 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 (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 UCHL1 in CSF and other bodily fluids can be used as a biomarker of neuronal injury or degeneration. The HGNC name for this protein is UCHL1.
• Storage Buffer: PBS, pH 7.4 with 0.02% sodium azide.
• Preparation and Storage: Store at -20 degree C/1 year

Western Blot (WB)

Testing Data #2

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).2. 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).3. 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).

NCBI and Uniprot Product Information

• NCBI GI #: 21361091
• NCBI GeneID: 7345
• NCBI Accession #: NP_004172.2
• NCBI GenBank Nucleotide #: NM_004181.4
• UniProt Accession #: P09936; Q4W5K6; Q71UM0
• Molecular Weight: 24,824 Da
• NCBI Official Full Name: ubiquitin carboxyl-terminal hydrolase isozyme L1
• NCBI Official Synonym Full Names: ubiquitin carboxyl-terminal esterase L1 (ubiquitin thiolesterase)
• NCBI Official Symbol: UCHL1
• NCBI Official Synonym Symbols: PARK5; PGP95; PGP9.5; Uch-L1; PGP 9.5
• NCBI Protein Information: ubiquitin carboxyl-terminal hydrolase isozyme L1; ubiquitin thioesterase L1; neuron cytoplasmic protein 9.5; ubiquitin C-terminal hydrolase
• UniProt Protein Name: Ubiquitin carboxyl-terminal hydrolase isozyme L1
• Protein Family: Ubiquitin carboxyl-terminal hydrolase
• UniProt Gene Name: UCHL1
• UniProt Synonym Gene Names: UCH-L1; PGP9.5
• UniProt Entry Name: UCHL1_HUMAN

NCBI Description

The protein encoded by this gene belongs to the peptidase C12 family. This enzyme is a thiol protease that hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. This gene is specifically expressed in the neurons and in cells of the diffuse neuroendocrine system. Mutations in this gene may be associated with Parkinson disease.[provided by RefSeq, Sep 2009]

Uniprot Description

Function: Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. This enzyme is a thiol protease that recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. Also binds to free monoubiquitin and may prevent its degradation in lysosomes. The homodimer may have ATP-independent ubiquitin ligase activity. Ref.12 Ref.13 Ref.28

Catalytic activity: Thiol-dependent hydrolysis of ester, thioester, amide, peptide and isopeptide bonds formed by the C-terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal). Ref.20

Subunit structure: Monomer. Homodimer. Interacts with SNCA

By similarity. Interacts with COPS5. Ref.14 Ref.19

Subcellular location: Cytoplasm. Endoplasmic reticulum membrane; Lipid-anchor. Note: About 30% of total UCHL1 is associated with membranes in brain. Ref.16

Tissue specificity: Found in neuronal cell bodies and processes throughout the neocortex (at protein level). Expressed in neurons and cells of the diffuse neuroendocrine system and their tumors. Weakly expressed in ovary. Down-regulated in brains from Parkinson disease and Alzheimer disease patients. Ref.5 Ref.12

Post-translational modification: O-glycosylated

By similarity.

Involvement in disease: Parkinson disease 5 (PARK5) [MIM:613643]: A complex neurodegenerative disorder with manifestations ranging from typical Parkinson disease to dementia with Lewy bodies. Clinical features include parkinsonian symptoms (resting tremor, rigidity, postural instability and bradykinesia), dementia, diffuse Lewy body pathology, autonomic dysfunction, hallucinations and paranoia.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.8 Ref.13 Ref.21 Ref.27

Miscellaneous: Oxidation of Met-1, Met-6, Met-12, Met-124 and Met-179 to methionine sulfoxide, and oxidation of Cys-220 to cysteine sulfonic acid have been observed in brains from Alzheimer disease (AD) and Parkinson disease (PD) patients. In AD, UCHL1 was found to be associated with neurofibrillary tangles. In contrast to UCHL3, does not hydrolyze a peptide bond at the C-terminal glycine of NEDD8.

Sequence similarities: Belongs to the peptidase C12 family.

Caution: Ref.8 reports the association of mutation Ile93Met with Parkinson disease. However, according to Ref.27 this association is uncertain and UCHL1 is not a susceptibility gene for Parkinson disease.

Biophysicochemical propertiesKinetic parameters:KM=122 nM for Ub-AMC Ref.8 Ref.11 Ref.21KM=1.20 µM for ubiquitin ethyl esterVmax=0.47 µmol/min/mg enzyme toward Ub-AMCVmax=25 µmol/min/mg enzyme toward ubiquitin ethyl ester

Sequence caution: The sequence CAA28443.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.