antibody

Anti-Ubiquitin C Terminal Hydrolase 1 (UCHL1)

MBS502135

0.1 ml

235 USD

monoclonal

mouse

nonconjugated

BH7

human, rat, cow

western blot, immunocytochemistry

Antibody

Anti-Ubiquitin C Terminal Hydrolase 1 (UCHL1)

Ubiquitin C-terminal Hydrolase 1 (UCHL1) ms

ubiquitin carboxyl-terminal hydrolase isozyme L1; Ubiquitin carboxyl-terminal hydrolase isozyme L1; ubiquitin carboxyl-terminal hydrolase isozyme L1; ubiquitin thioesterase L1; epididymis luminal protein 117; neuron cytoplasmic protein 9.5; ubiquitin C-terminal hydrolase; ubiquitin carboxyl-terminal esterase L1 (ubiquitin thiolesterase); Neuron cytoplasmic protein 9.5; PGP 9.5; PGP9.5; Ubiquitin thioesterase L1

UCHL1

UCHL1; UCHL1; NDGOA; PARK5; PGP95; PGP9.5; Uch-L1; HEL-117; PGP 9.5; UCH-L1; PGP9.5

UCHL1_HUMAN

Monoclonal

IgG1

BH7

Mouse

Bovine, human, rat

223

Specific for the ~24kDa UCHL1 protein.

Total IgG fraction (Unpurified, concentrated culture supernatant.)

Liquid. Contains 10 mM sodium azide.

Store at -20 degree C in undiluted aliquots; stable for at least 1 year. Avoid freeze/thaw cycles.

Western Blot (WB), Immunofluorescence (IF)

Quality Control: Western blots performed on each lot. WB: 1:5,000. IF: 1:500

Antigen: Recombinant full length human UCHL1 purified from E. coli. Immunogen Information: Recombinant full length human UCHL1 purified from E. coli. Immunogen Species: Human

Reactivity Assumed Based on 100% Sequence Homology: Most mammalian. Species Reactivity Note: The antibody has been directly tested for reactivity in bovine, human, mouse and rat. It is expected that it will work on other mammalian tissues. Biological Significance: 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 (1). It was later found that ubiquitin C-terminal hydrolase enzyme activity was associated with the PGP9.5 protein (2). 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 (3). Point mutations in the UCHL1 gene are associated with some forms of human Parkinson's disease (4). 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.

Mouse monoclonal antibody

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). 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).

21361091

NP_004172.2

NM_004181.4

P09936

24

Alpha-synuclein Signaling Pathway 137913!!Parkinson's Disease Pathway 83098!!Parkinsons Disease Pathway 705377!!Proteasome Degradation Pathway 198786

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]

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 uM for ubiquitin ethyl esterVmax=0.47 umol/min/mg enzyme toward Ub-AMCVmax=25 umol/min/mg enzyme toward ubiquitin ethyl ester. Sequence caution: The sequence CAA28443.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.

0.1 ml

235 USD