Cloned (Comment) | Organism |
---|---|
UCHL1 gene, the gene and its proximal promoter with a classic TATA box 25 bp upstream of a G base determined to be the transcription start site from mRNA 5'-mapping analyses, gene ragulation, detailed overview | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
I93M | naturally occuring mutation involved in Parkinson's disease | Homo sapiens |
additional information | isolated loss of UCHL1/PGP 9.5 function, seen in the gracile axonal dystrophy, GAD, mouse due to a deletion in its gene results in a failure of axonal transport and a dying-back axonopathy beginning distally in long axons, the characteristic lesion in the GAD mouse is axonal dystrophy, gad mouse neuronal function phenotype, detailed overview | Mus musculus |
S18Y | naturally occuring mutation involved in Parkinson's disease | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
cytoplasm | major enzyme portion of UCHL1/PGP 9.5 | Mus musculus | 5737 | - |
cytoplasm | major enzyme portion of UCHL1/PGP 9.5 | Homo sapiens | 5737 | - |
cytoplasm | major enzyme portion of UCHL1/PGP 9.5 | Rattus norvegicus | 5737 | - |
cytoplasm | major enzyme portion of UCHL1/PGP 9.5 | Bos taurus | 5737 | - |
cytoplasm | major enzyme portion of UCHL1/PGP 9.5 | Macaca fuscata | 5737 | - |
membrane | minor enzyme portion of UCHL1/PGP 9.5, tightly bound | Mus musculus | 16020 | - |
membrane | minor enzyme portion of UCHL1/PGP 9.5, tightly bound | Homo sapiens | 16020 | - |
membrane | minor enzyme portion of UCHL1/PGP 9.5, tightly bound | Rattus norvegicus | 16020 | - |
membrane | minor enzyme portion of UCHL1/PGP 9.5, tightly bound | Macaca fuscata | 16020 | - |
membrane | minor enzyme portion of UCHL1/PGP 9.5, tightly bound, UCHL3 | Bos taurus | 16020 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Homo sapiens | polyubiquitin processing, overview. UCHL1/PGP 9.5 also shows ligase activity when in a dimeric form, overview | ? | - |
? | |
polyubiquitin + H2O | Homo sapiens | substrate of UCHL3, no activity with UCHL1/PGP 9.5 | ubiquitin | - |
? | |
polyubiquitin + H2O | Bos taurus | substrate of UCHL3, no activity with UCHL1/PGP 9.5 | ubiquitin | - |
? | |
polyubiquitin + H2O | Mus musculus | UCHL1/PGP 9.5, overview | ubiquitin | - |
? | |
ubiquitin-CEP52 + H2O | Homo sapiens | substrate of UCHL1/PGP 9.5, no activity with UCHL3 | ubiquitin + CEP52 | - |
? | |
ubiquitin-CEP52 + H2O | Bos taurus | substrate of UCHL1/PGP 9.5, no activity with UCHL3 | ubiquitin + CEP52 | - |
? | |
ubiquitin-CEP80 + H2O | Homo sapiens | substrate of UCHL3 and UCHL1/PGP 9.5 | ubiquitin + CEP80 | - |
? | |
ubiquitin-CEP80 + H2O | Bos taurus | substrate of UCHL3 and UCHL1/PGP 9.5 | ubiquitin + CEP80 | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Bos taurus | - |
three isozymes UCHL1, UCHL2, and UCHL3 | - |
Homo sapiens | - |
- |
- |
Macaca fuscata | - |
- |
- |
Mus musculus | - |
- |
- |
Rattus norvegicus | - |
- |
- |
Purification (Comment) | Organism |
---|---|
native isozymes UCHL1-L3 from thymus by anion exchange chromatography and gel filtration, isozyme UCHL1 to homogeneity after this step. PGP 9.5 purified from bovine brain, isozyme UCHL3 is further specifically purified by ubiquitin affinity chromatography | Bos taurus |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
astrocyte | - |
Homo sapiens | - |
brain | - |
Mus musculus | - |
brain | - |
Rattus norvegicus | - |
brain | high content of UCHL1 | Macaca fuscata | - |
brain | high content of UCHL1, distribution in brain tissues, overview | Homo sapiens | - |
brain | PGP 9.5 represents a ubiquitin carboxy-terminal hydrolase highly, localized in nervous tissue | Bos taurus | - |
epidermis | of mouse hind-paw | Mus musculus | - |
epidermis | of mouse hind-paw | Rattus norvegicus | - |
glial cell | - |
Homo sapiens | - |
glioma cell | - |
Homo sapiens | - |
kidney | - |
Homo sapiens | - |
large intestine | - |
Homo sapiens | - |
additional information | immunohistochemic analysis, overview | Rattus norvegicus | - |
additional information | tissue distribution and immunohistochemic analysis, overview | Mus musculus | - |
additional information | tissue distribution and immunohistochemic analysis, overview | Rattus norvegicus | - |
additional information | tissue distribution and immunohistochemic analysis, overview | Bos taurus | - |
additional information | UCHL1/PGP 9.5 is present prominently in brain, in addition to its neuronal localization, UCHL1/PGP 9.5 is also present in cells of the diffuse neuroendocrine system, traces of the protein are also seen in large intestine, kidney, ovary, and testis. Tissue distribution and immunohistochemic analysis, overview. Enzyme activity in the diffuse neuroendocrine system, e.g. in gastroenteropancreatic system, prostate, pulmonary neuroendocrine system, and cerebral cortex, detailed overview | Homo sapiens | - |
neuroendocrine cell | high content of UCHL1 | Mus musculus | - |
neuroendocrine cell | high content of UCHL1 | Rattus norvegicus | - |
neuroendocrine cell | high content of UCHL1. UCHL1/PGP 9.5 is a highly conserved protein present in virtually all neurones and neuroendocrine cells as a major component of soluble cytoplasmic protein | Homo sapiens | - |
neuroendocrine cell | high content of UCHL1. UCHL1/PGP 9.5 is a highly conserved protein present in virtually all neurones and neuroendocrine cells as a major component of soluble cytoplasmic protein | Macaca fuscata | - |
neuron | high content of UCHL1, distribution in neuron types and cellular compartments, overview | Mus musculus | - |
neuron | high content of UCHL1, distribution in neuron types and cellular compartments, overview | Rattus norvegicus | - |
neuron | high content of UCHL1. UCHL1/PGP 9.5 is a highly conserved protein present in virtually all neurones and neuroendocrine cells as a major component of soluble cytoplasmic protein | Homo sapiens | - |
neuron | high content of UCHL1. UCHL1/PGP 9.5 is a highly conserved protein present in virtually all neurones and neuroendocrine cells as a major component of soluble cytoplasmic protein | Macaca fuscata | - |
olfactory epithelium | - |
Mus musculus | - |
olfactory epithelium | - |
Rattus norvegicus | - |
olfactory receptor neuron | distribution in cellular compartments, overview | Mus musculus | - |
olfactory receptor neuron | the olfactory receptor neurones (replaceable) appear to be strongly immunoreactive as do the principal neurones (mitral and mitral/tufted cells), while in the olfactory bulb the replaceable periglomerular and granule cells appear to be non-reactive, distribution in cellular compartments, overview | Rattus norvegicus | - |
oocyte | - |
Homo sapiens | - |
oocyte | UCHL1 and UCHL3 are present in the oocyte throughout the oestrous cycle in wild-type mice, but appropriately immunoreactivity for UCHL1 is absent from the gad mouse and UCHL3 immunoreactivity is absent from the UCHL3 knock-out mouse, overview | Mus musculus | - |
ovary | strong cytoplasmic staining of ova in primordial and developing follicles with moderate staining of the theca externa and the corpus luteum | Homo sapiens | - |
retina | photoreceptor cells, bipolar cells, and amacrine cells are devoid of staining while the dendrites and axons of both the horizontal cells and the ganglion cells stain strongly | Homo sapiens | - |
retina | UCHL1/PGP 9.5, photoreceptor cells, bipolar cells, and amacrine cells are devoid of staining while the dendrites and axons of both the horizontal cells and the ganglion cells stain strongly | Rattus norvegicus | - |
retina | UCHL1/PGP 9.5, photoreceptor cells, bipolar cells, and amacrine cells are devoid of staining while the dendrites and axons of both the horizontal cells and the ganglion cells stain strongly | Bos taurus | - |
spermatogonium | spermatogonia in humans can be subdivided into three types, overview | Homo sapiens | - |
spermatogonium | spermatogonia in monkeys can be subdivided into three types, overview | Macaca fuscata | - |
testis | - |
Homo sapiens | - |
thymus | - |
Bos taurus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | polyubiquitin processing, overview. UCHL1/PGP 9.5 also shows ligase activity when in a dimeric form, overview | Homo sapiens | ? | - |
? | |
additional information | purified PGP 9.5 from bovine brain exhibits a specific activity in cleaving ubiquitin-ethylester which is 40% of that shown by bovine thymus UCHL3. UCHL1/PGP 9.5 and UCHL3 hydrolysis rates of various synthetic ubiquitin conjugates compare to rates of hydrolysis of the generic substrate ubiquitin-ethylester. Both enzymes cleave ubiquitin from N-epsilon linked lysine or N-alpha-linked lysine equally well. Neither can cleave the Ub-K48 Ub bond, but both can cleave lysine from the C-terminal of this conjugate. UCHL3 can readily cleave a wide variety of peptides from the C-terminus of ubiquitin if the peptides are less than 20 residues, UCHL1/PGP 9.5 is at least a hundred times less active in this | Bos taurus | ? | - |
? | |
additional information | the apparent catalytic triad of amino acids in UCHL1 is Cys90, His161 and Asp176 in the active site. The region forming a disordered crossover loop limiting access of substrates to the active site is located at positions 140-160, overview | Homo sapiens | ? | - |
? | |
polyubiquitin + H2O | - |
Mus musculus | ubiquitin | - |
? | |
polyubiquitin + H2O | substrate of UCHL3, no activity with UCHL1/PGP 9.5 | Homo sapiens | ubiquitin | - |
? | |
polyubiquitin + H2O | substrate of UCHL3, no activity with UCHL1/PGP 9.5 | Bos taurus | ubiquitin | - |
? | |
polyubiquitin + H2O | UCHL1/PGP 9.5, overview | Mus musculus | ubiquitin | - |
? | |
ubiquitin C-terminal-4-methyl-7-amido-coumarin + H2O | UCHL3 is 200fold more active than UCHL1/PGP 9.5 | Bos taurus | ubiquitin + 4-methyl-7-amino-coumarin | - |
? | |
ubiquitin ethylester + H2O | synthetic substrate | Bos taurus | ubiquitin + an alcohol | - |
? | |
ubiquitin-CEP52 + H2O | substrate of UCHL1/PGP 9.5, no activity with UCHL3 | Homo sapiens | ubiquitin + CEP52 | - |
? | |
ubiquitin-CEP52 + H2O | substrate of UCHL1/PGP 9.5, no activity with UCHL3 | Bos taurus | ubiquitin + CEP52 | - |
? | |
ubiquitin-CEP80 + H2O | substrate of UCHL3 and UCHL1/PGP 9.5 | Homo sapiens | ubiquitin + CEP80 | - |
? | |
ubiquitin-CEP80 + H2O | substrate of UCHL3 and UCHL1/PGP 9.5 | Bos taurus | ubiquitin + CEP80 | - |
? |
Synonyms | Comment | Organism |
---|---|---|
PGP 9.5 | - |
Mus musculus |
PGP 9.5 | - |
Homo sapiens |
PGP 9.5 | - |
Rattus norvegicus |
PGP 9.5 | - |
Bos taurus |
PGP 9.5 | - |
Macaca fuscata |
ubiquitin carboxyl-terminal hydrolase | - |
Bos taurus |
UCHL1 | - |
Mus musculus |
UCHL1 | - |
Homo sapiens |
UCHL1 | - |
Rattus norvegicus |
UCHL1 | - |
Bos taurus |
UCHL1 | - |
Macaca fuscata |
UCHL1/PGP 9.5 | - |
Mus musculus |
UCHL1/PGP 9.5 | - |
Homo sapiens |
UCHL1/PGP 9.5 | - |
Rattus norvegicus |
UCHL1/PGP 9.5 | - |
Bos taurus |
UCHL1/PGP 9.5 | - |
Macaca fuscata |
UCHL2 | - |
Bos taurus |
Uchl3 | - |
Bos taurus |
Organism | Comment | Expression |
---|---|---|
Homo sapiens | aberrant expression of UCHL1/PGP 9.5 in Schwann cells after nerve transection and even in fibroblasts after skin wounds | down |
General Information | Comment | Organism |
---|---|---|
malfunction | human UCHL1 gene and neurological diseases, wild-type and mutants I93M and S18Y, mutation I93M is proposed as a familial Parkinsons disease mutation, PARK5, detailed overview | Homo sapiens |
metabolism | UCHL1 gene regulation, overview | Homo sapiens |
physiological function | UCHL1/PGP 9.5 is important in the ubiquitin system, and functions of UCHL1/PGP 9.5 in neurones, detailed overview | Homo sapiens |
physiological function | UCHL1/PGP 9.5 is important in the ubiquitin system, detailed overview | Rattus norvegicus |
physiological function | UCHL1/PGP 9.5 is important in the ubiquitin system, detailed overview | Bos taurus |
physiological function | UCHL1/PGP 9.5 is important in the ubiquitin system, detailed overview. UCHL1/PGP 9.5 might be involved in blocking polyspermy, particularly at the plasma membrane | Mus musculus |