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Literature summary for 3.4.19.12 extracted from

  • Day, I.N.; Thompson, R.J.
    UCHL1 (PGP 9.5): neuronal biomarker and ubiquitin system protein (2010), Prog. Neurobiol., 90, 327-362.
    View publication on PubMed

Cloned(Commentary)

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

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

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/ Products (Substrates)

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

Organism UniProt Comment Textmining
Bos taurus
-
three isozymes UCHL1, UCHL2, and UCHL3
-
Homo sapiens
-
-
-
Macaca fuscata
-
-
-
Mus musculus
-
-
-
Rattus norvegicus
-
-
-

Purification (Commentary)

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

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 and Products (Substrate)

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

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

Expression

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

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 Parkinson’s 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