UCHL1

Summary

Gene Symbol: UCHL1
Description: ubiquitin C-terminal hydrolase L1
Alias: HEL-117, HEL-S-53, NDGOA, PARK5, PGP 9.5, PGP9.5, PGP95, SPG79, Uch-L1, ubiquitin carboxyl-terminal hydrolase isozyme L1, epididymis luminal protein 117, epididymis secretory protein Li 53, neuron cytoplasmic protein 9.5, ubiquitin carboxyl-terminal esterase L1 (ubiquitin thiolesterase), ubiquitin thioesterase L1, ubiquitin thiolesterase
Species: human

Top Publications

  1. ncbi Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice
    K Saigoh
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, 187 8502, Japan
    Nat Genet 23:47-51. 1999
  2. pmc Aberrant interaction between Parkinson disease-associated mutant UCH-L1 and the lysosomal receptor for chaperone-mediated autophagy
    Tomohiro Kabuta
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4 1 1 Ogawahigashi, Kodaira, Tokyo, Japan
    J Biol Chem 283:23731-8. 2008
  3. pmc Structural basis for conformational plasticity of the Parkinson's disease-associated ubiquitin hydrolase UCH-L1
    Chittaranjan Das
    Department of Chemistry and Biochemistry, Rosenstiel Basic Medical Sciences Research Center, Brandeis University, Waltham, MA 02454 9110, USA
    Proc Natl Acad Sci U S A 103:4675-80. 2006
  4. ncbi Human prostate cancer cells express neuroendocrine cell markers PGP 9.5 and chromogranin A
    Aaron Leiblich
    Section of Oncology, Academic Urology Unit, University of Sheffield, Royal Hallamshire Hospital, Sheffield, UK
    Prostate 67:1761-9. 2007
  5. doi Ubiquitin C-terminal hydrolase-L1 interacts with adhesion complexes and promotes cell migration, survival, and anchorage independent growth
    Teresa Frisan
    Department of Cell and Molecular Biology, Karolinska Institutet, Stockholm, Sweden, Box 285, S 171 77 Stockholm, Sweden
    FASEB J 26:5060-70. 2012
  6. doi Prognostic relevance of ubiquitin C-terminal hydrolase L1 (UCH-L1) mRNA and protein expression in breast cancer patients
    Christine Schröder
    Department of Gynaecology, University Medical Centre Hamburg Eppendorf, Hamburg, Germany
    J Cancer Res Clin Oncol 139:1745-55. 2013
  7. ncbi UCHL-1 gene in multiple system atrophy: a haplotype tagging approach
    Daniel G Healy
    Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London United Kingdom
    Mov Disord 20:1338-43. 2005
  8. ncbi DJ-1 and UCH-L1 gene activity patterns in the brains of controls, Parkinson and schizophrenia patients and in rodents
    Dagmar Galter
    Karolinska Institutet, Department of Neuroscience, Retzius väg 8 B2 4, S 171 77 Stockholm, Sweden
    Physiol Behav 92:46-53. 2007
  9. pmc Neuronal and glial markers are differently associated with computed tomography findings and outcome in patients with severe traumatic brain injury: a case control study
    Stefania Mondello
    Department of Anesthesiology, University of Florida, 1600 S, W, Archer Road, Gainesville, FL 32610 0254, USA
    Crit Care 15:R156. 2011
  10. ncbi Isolation of PGP 9.5, a new human neurone-specific protein detected by high-resolution two-dimensional electrophoresis
    J F Doran
    J Neurochem 40:1542-7. 1983

Research Grants

  1. Pembe Hande Ozdinler; Fiscal Year: 2016
  2. High Throughout Assay to Probe UCH-L1 Ligase Inhibitors
    Peter Lansbury; Fiscal Year: 2005
  3. Structure and Mechanism of the Parkin Ubiquitin Ligase
    Brenda Schulman; Fiscal Year: 2003
  4. Molecular Epidemiology of Parkinson's Disease
    DEMETRIUS MICHAEL MARAGANORE; Fiscal Year: 2010
  5. role of ubiquitinin in cancer progression
    Jin Ming Yang; Fiscal Year: 2005
  6. Conditional Uch-L1 knockout mice
    Michael Lee; Fiscal Year: 2005
  7. MOLECULAR MECHANISMS OF THE PARKINSON'S DISEASE GENES
    Asa Abeliovich; Fiscal Year: 2007
  8. Amy L Wilson-Delfosse; Fiscal Year: 2014
  9. Role of UCH-L1 Oxidative Modification in Parkinson's Disease Pathogenesis
    JEANNE ELIZABETH MCKEON; Fiscal Year: 2012
  10. Steven H Graham; Fiscal Year: 2016

Detail Information

Publications201 found, 100 shown here

  1. ncbi Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice
    K Saigoh
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, 187 8502, Japan
    Nat Genet 23:47-51. 1999
    ..Here we find that the gad mutation is caused by an in-frame deletion including exons 7 and 8 of Uchl1, encoding the ubiquitin carboxy-terminal hydrolase (UCH) isozyme (Uch-l1) selectively expressed in the nervous ..
  2. pmc Aberrant interaction between Parkinson disease-associated mutant UCH-L1 and the lysosomal receptor for chaperone-mediated autophagy
    Tomohiro Kabuta
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4 1 1 Ogawahigashi, Kodaira, Tokyo, Japan
    J Biol Chem 283:23731-8. 2008
    ....
  3. pmc Structural basis for conformational plasticity of the Parkinson's disease-associated ubiquitin hydrolase UCH-L1
    Chittaranjan Das
    Department of Chemistry and Biochemistry, Rosenstiel Basic Medical Sciences Research Center, Brandeis University, Waltham, MA 02454 9110, USA
    Proc Natl Acad Sci U S A 103:4675-80. 2006
    ..In particular, the geometry of the catalytic residues in the active site of UCH-L1 is distorted in such a way that the hydrolytic activity would appear to be impossible without substrate induced conformational rearrangements...
  4. ncbi Human prostate cancer cells express neuroendocrine cell markers PGP 9.5 and chromogranin A
    Aaron Leiblich
    Section of Oncology, Academic Urology Unit, University of Sheffield, Royal Hallamshire Hospital, Sheffield, UK
    Prostate 67:1761-9. 2007
    ..To identify novel candidates associated with metastatic progression, we compared the proteomic profiles of LNCaP (lymph-node metastatic, androgen-dependant) and PC-3 (bone metastatic, androgen-independent), human prostate cancer cells...
  5. doi Ubiquitin C-terminal hydrolase-L1 interacts with adhesion complexes and promotes cell migration, survival, and anchorage independent growth
    Teresa Frisan
    Department of Cell and Molecular Biology, Karolinska Institutet, Stockholm, Sweden, Box 285, S 171 77 Stockholm, Sweden
    FASEB J 26:5060-70. 2012
    ..These findings offer new insights on the molecular interactions through which the deubiquitinating enzyme regulates the survival, proliferation, and metastatic potential of malignant cells...
  6. doi Prognostic relevance of ubiquitin C-terminal hydrolase L1 (UCH-L1) mRNA and protein expression in breast cancer patients
    Christine Schröder
    Department of Gynaecology, University Medical Centre Hamburg Eppendorf, Hamburg, Germany
    J Cancer Res Clin Oncol 139:1745-55. 2013
    ..However, only limited information about its role in breast cancer is available. The aim of this study was to examine the UCH-L1 expression in primary breast cancer and to determine its relevance as a potential prognostic marker...
  7. ncbi UCHL-1 gene in multiple system atrophy: a haplotype tagging approach
    Daniel G Healy
    Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London United Kingdom
    Mov Disord 20:1338-43. 2005
    ..This search included the S18Y variant of UCHL-1, which has been reported to be protective in Parkinson's disease...
  8. ncbi DJ-1 and UCH-L1 gene activity patterns in the brains of controls, Parkinson and schizophrenia patients and in rodents
    Dagmar Galter
    Karolinska Institutet, Department of Neuroscience, Retzius väg 8 B2 4, S 171 77 Stockholm, Sweden
    Physiol Behav 92:46-53. 2007
    ..The abundant expression of DJ-1 in certain peripheral tissues and of UCH-L1 in peripheral neurons may also be of relevance for the spectrum of symptoms in different forms of PD...
  9. pmc Neuronal and glial markers are differently associated with computed tomography findings and outcome in patients with severe traumatic brain injury: a case control study
    Stefania Mondello
    Department of Anesthesiology, University of Florida, 1600 S, W, Archer Road, Gainesville, FL 32610 0254, USA
    Crit Care 15:R156. 2011
    ....
  10. ncbi Isolation of PGP 9.5, a new human neurone-specific protein detected by high-resolution two-dimensional electrophoresis
    J F Doran
    J Neurochem 40:1542-7. 1983
    ..This new neurone-specific cytoplasmic marker may prove useful in studies of neuronal development and in the detection of neuronal damage in disease of the nervous system...
  11. pmc Lack of evidence for an association between UCHL1 S18Y and Parkinson's disease
    Carolyn M Hutter
    Department of Epidemiology, University of Washington, Seattle, WA, USA
    Eur J Neurol 15:134-9. 2008
    b>UCHL1 has been proposed as a candidate gene for Parkinson's disease (PD). A meta-analysis of white and Asian subjects reported an inverse association between the non-synonymous UCHL1 S18Y polymorphism and PD risk...
  12. pmc Regulation of synaptic structure by ubiquitin C-terminal hydrolase L1
    Anna E Cartier
    Section of Neurobiology, Department of Biological Sciences, University of California, San Diego, La Jolla, California 92093 0347, USA
    J Neurosci 29:7857-68. 2009
    ..These findings point to a significant role of UCH-L1 in synaptic remodeling, most likely by modulating free monomeric ubiquitin levels in an activity-dependent manner...
  13. ncbi Genetic association between Ubiquitin Carboxy-terminal Hydrolase-L1 gene S18Y polymorphism and sporadic Alzheimer's disease in a Chinese Han population
    Sufang Xue
    Department of Neurology, Xuanwu Hospital, Capital University of Medical Sciences, 45 Changchun Street, 100053 Beijing, China
    Brain Res 1087:28-32. 2006
    ..We conclude that Y allele and YY genotype of S18Y in the UCH-L1 gene may have a protective effect against sporadic AD in female subjects, probably due to altering the function of UCH-L1 and the interactions among different risk factors...
  14. ncbi Silencing of the UCHL1 gene in human colorectal and ovarian cancers
    Eriko Okochi-Takada
    Carcinogenesis Division, National Cancer Center Research Institute, Tokyo, Japan
    Int J Cancer 119:1338-44. 2006
    ..Among these was the UCHL1 gene (also known as PGP9.5), which is involved in regulation of cellular ubiquitin levels...
  15. ncbi Evidence for a role of the ubiquitin-proteasome pathway in pancreatic islets
    Maria D Lopez-Avalos
    Section on Islet Transplantation and Cell Biology, Joslin Diabetes Center, Harvard Medical School, Boston, MA, USA
    Diabetes 55:1223-31. 2006
    ....
  16. ncbi Altered gene expression in cells from patients with lysosomal storage disorders suggests impairment of the ubiquitin pathway
    P Bifsha
    Sainte Justine Hospital, Department of Pediatrics and Biochemistry, University of Montreal, Montreal, Canada
    Cell Death Differ 14:511-23. 2007
    ....
  17. doi Analysis of the UCHL1 genetic variant in Parkinson's disease among Chinese
    E K Tan
    Department of Neurology, Singapore General Hospital, National Neuroscience Institute, Singapore
    Neurobiol Aging 31:2194-6. 2010
    The inverse association of the functional ubiquitin carboxy-terminal hydrolase L1 (UCHL1) S18Y variant with Parkinson's disease (PD) among Caucasian populations has been debated...
  18. ncbi ACT and UCH-L1 polymorphisms in Parkinson's disease and age of onset
    Jian Wang
    Institute of Genetics, School of Life Science, Fudan University, Shanghai, People s Republic of China
    Mov Disord 17:767-71. 2002
    ..557 (95% C.I. = 0.314-0.985; P = 0.043). These data suggest that ACT polymorphism does not influence the risk for developing PD. UCH-L1 S18Y polymorphism, however, may be a weak protective factor against early-onset PD...
  19. ncbi S18Y polymorphism in the UCH-L1 gene and Parkinson's disease: evidence for an age-dependent relationship
    Alexis Elbaz
    Institut National de la Sante et de la Recherche Medicale, Unit 360, Hopital de la Salpetriere, Paris, France
    Mov Disord 18:130-7. 2003
    ..In this community-based case-control study, carriers of the Y allele were at decreased risk of developing PD at a young age, independently of pesticides and smoking...
  20. ncbi Reduced ubiquitin C-terminal hydrolase-1 expression levels in dementia with Lewy bodies
    Marta Barrachina
    Institut de Neuropatologia, Servei Anatomia Patològica, IDIBELL Hospital Universitari de Bellvitge, L Hospitalet de Llobregat, Spain
    Neurobiol Dis 22:265-73. 2006
    ..Together, these observations show reduced UCHL-1 expression as a contributory factor in the abnormal protein aggregation in DLB, and points UCHL-1 as a putative therapeutic target in the treatment of DLB...
  21. ncbi Ubiquitin COOH-terminal hydrolase 1: a biomarker of renal cell carcinoma associated with enhanced tumor cell proliferation and migration
    Barbara Seliger
    Institute of Medical Immunology, Martin Luther University Halle Wittenberg, Halle, Germany
    Clin Cancer Res 13:27-37. 2007
    ..Due to the currently inefficient therapies and the low 5-year survival rates of RCC patients, novel diagnostic, prognostic, and therapeutic markers are urgently needed for this disease...
  22. ncbi PGP9.5 methylation as a marker for metastatic colorectal cancer
    Hiroki Mizukami
    Department of Surgery, Showa University Fujigaoka Hospital, Yokohama 227 8501, Japan
    Anticancer Res 28:2697-700. 2008
    ..5 expression is related to tumor progression and may be useful as a marker for invasive colorectal cancer. These results prompted us to examine the methylation status of the PGP9.5 gene in colorectal cancer...
  23. ncbi The gene for human neurone specific ubiquitin C-terminal hydrolase (UCHL1, PGP9.5) maps to chromosome 4p14
    Y H Edwards
    M R C Human Biochemical Genetics Unit, Galton Laboratory, London
    Ann Hum Genet 55:273-8. 1991
    Ubiquitin carboxy terminal hydrolase 1, UCHL1, is a neurone-specific protein involved in the ubiquitin-mediated proteolytic pathway...
  24. pmc The structure of the human gene encoding protein gene product 9.5 (PGP9.5), a neuron-specific ubiquitin C-terminal hydrolase
    I N Day
    University Department of Clinical Biochemistry, Southampton General Hospital, U K
    Biochem J 268:521-4. 1990
    ..The human PGP9.5 gene, described here, spans 10 kb, contains nine exons and displays 5' features some common to many genes and some common with neurofilament neuron-specific enolase and Thy-1-antigen gene 5' regions...
  25. ncbi Molecular cloning of cDNA coding for human PGP 9.5 protein. A novel cytoplasmic marker for neurones and neuroendocrine cells
    I N Day
    FEBS Lett 210:157-60. 1987
    ..Protein sequencing of 50% of PGP 9.5 confirms the deduced protein sequence...
  26. ncbi Substrate binding and catalysis by ubiquitin C-terminal hydrolases: identification of two active site residues
    C N Larsen
    Department of Biochemistry, Emory University, Atlanta, Georgia 30322, USA
    Biochemistry 35:6735-44. 1996
    ..6 microM). The binding is primarily electrostatic in nature and indicates the existence of a specific and extensive binding site for ubiquitin on the surface of the enzyme...
  27. ncbi Substrate specificity of deubiquitinating enzymes: ubiquitin C-terminal hydrolases
    C N Larsen
    Department of Biochemistry, Emory University, Atlanta, Georgia 30322, USA
    Biochemistry 37:3358-68. 1998
    ....
  28. ncbi The ubiquitin pathway in Parkinson's disease
    E Leroy
    Nature 395:451-2. 1998
  29. ncbi Low frequency of pathogenic mutations in the ubiquitin carboxy-terminal hydrolase gene in familial Parkinson's disease
    S Lincoln
    Mayo Clinic Jacksonville, FL 32224, USA
    Neuroreport 10:427-9. 1999
    ..These changes are, therefore, unlikely to be pathogenic. We conclude that the I93M variant must either be a rare cause of disease or a harmless substitution whose occurrence in the family reflects a chance co-occurrence...
  30. ncbi The Ile93Met mutation in the ubiquitin carboxy-terminal-hydrolase-L1 gene is not observed in European cases with familial Parkinson's disease
    B S Harhangi
    Department of Epidemiology and Biostatistics, Erasmus University Medical School, Rotterdam, The Netherlands
    Neurosci Lett 270:1-4. 1999
    ..We conclude that the UCH-L1 gene is not a major gene responsible for familial PD...
  31. ncbi A polymorphic variation of serine to tyrosine at codon 18 in the ubiquitin C-terminal hydrolase-L1 gene is associated with a reduced risk of sporadic Parkinson's disease in a Japanese population
    J Satoh
    Division of Neurology, Department of Internal Medicine, Saga Medical School, 5 1 1 Nabeshima, Saga 849 8501, Japan
    J Neurol Sci 189:113-7. 2001
    ....
  32. ncbi Interaction and colocalization of PGP9.5 with JAB1 and p27(Kip1)
    Otavia L Caballero
    Department of Otolaryngology Head and Neck Surgery, Division of Head and Neck Cancer Research, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Oncogene 21:3003-10. 2002
    ..5 and JAB1 became more perinuclear and cytoplasmic in localization while p27(Kip1) was present only in the nucleus. Therefore, PGP9.5 may contribute to p27(Kip1) degradation via its interaction and nuclear translocation with JAB1...
  33. ncbi Mutation analysis and association studies of the ubiquitin carboxy-terminal hydrolase L1 gene in Huntington's disease
    Peggy Nazé
    Laboratoire de Biochimie et de Biologie Moleculaire, Unité Fonctionnelle de Neurobiologie, Hopital R Salengro, Centre Hospitalier Regional et Universitaire de Lille, 59037 Lille Cedex, France
    Neurosci Lett 328:1-4. 2002
    ..The S18Y polymorphism was found in three out of the four patients presenting with a later age-at-onset. We conclude that the UCH-L1 gene may be a genetic factor that influences the variability in age-at-onset of HD...
  34. ncbi Proteomic identification of oxidatively modified proteins in Alzheimer's disease brain. Part I: creatine kinase BB, glutamine synthase, and ubiquitin carboxy-terminal hydrolase L-1
    Alessandra Castegna
    Department of Chemistry, Center of Membrane Sciences, University of Kentucky, Lexington 40506 0055, USA
    Free Radic Biol Med 33:562-71. 2002
    ..Proteomics offers a rapid means of identifying oxidatively modified proteins in aging and age-related neurodegenerative disorders without the limitations of the immunochemical detection method...
  35. ncbi The UCH-L1 gene encodes two opposing enzymatic activities that affect alpha-synuclein degradation and Parkinson's disease susceptibility
    Yichin Liu
    Center for Neurologic Diseases, Brigham and Women s Hospital, Department of Neurology, Harvard Medical School, Cambridge, MA 02139, USA
    Cell 111:209-18. 2002
    ..Thus, the ligase activity as well as the hydrolase activity of UCH-L1 may play a role in proteasomal protein degradation, a critical process for neuronal health...
  36. ncbi Alterations of structure and hydrolase activity of parkinsonism-associated human ubiquitin carboxyl-terminal hydrolase L1 variants
    Kaori Nishikawa
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4 1 1 Ogawahigashi, Kodaira, 187 8502, Tokyo, Japan
    Biochem Biophys Res Commun 304:176-83. 2003
    ..Taken together, these results suggest that further studies of altered UCH-L1 hydrolase function may provide new insights into a possible common pathogenic mechanism between familial and sporadic Parkinson's disease...
  37. ncbi Ubiquitin carboxy-terminal hydrolase L1 binds to and stabilizes monoubiquitin in neuron
    Hitoshi Osaka
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, 187 8502, Japan
    Hum Mol Genet 12:1945-58. 2003
    Mammalian neuronal cells abundantly express a deubiquitylating enzyme, ubiquitin carboxy-terminal hydrolase 1 (UCH L1). Mutations in UCH L1 are linked to Parkinson's disease as well as gracile axonal dystrophy (gad) in mice...
  38. ncbi Oxidative modifications and down-regulation of ubiquitin carboxyl-terminal hydrolase L1 associated with idiopathic Parkinson's and Alzheimer's diseases
    Joungil Choi
    Department of Pharmacology, Center for Neurodegenerative Disease, Emory University School of Medicine, 1510 Clifton Road, Atlanta, GA 30322, USA
    J Biol Chem 279:13256-64. 2004
    ..Together, these results provide evidence supporting a direct link between oxidative damage to the neuronal ubiquitination/de-ubiquitination machinery and the pathogenesis of sporadic AD and PD...
  39. ncbi UCHL1 is a Parkinson's disease susceptibility gene
    Demetrius M Maraganore
    Department of Neurology, Mayo Clinic, Rochester, MN, USA
    Ann Neurol 55:512-21. 2004
    The reported inverse association between the S18Y variant of the ubiquitin carboxy-terminal hydrolase L1 (UCHL1) gene and Parkinson's disease (PD) has strong biological plausibility...
  40. ncbi UCHL-1 is not a Parkinson's disease susceptibility gene
    Daniel G Healy
    Department of Molecular Neuroscience, Institute of Neurology, University College London, London, United Kingdom
    Ann Neurol 59:627-33. 2006
    ..The strongest evidence comes from a meta-analysis of small studies that reported the S18Y polymorphism as protective against PD, after pooling studies of white and Asian subjects. Here, we present data that challenge this association...
  41. ncbi Up-regulation of expression of the ubiquitin carboxyl-terminal hydrolase L1 gene in human airway epithelium of cigarette smokers
    Brendan J Carolan
    Department of Genetic Medicine and Division of Pulmonary and Critical Care Medicine, Weill Medical College of Cornell University, New York, New York, USA
    Cancer Res 66:10729-40. 2006
    ..Of 11 genes considered to be neuroendocrine cell specific, only ubiquitin carboxyl-terminal hydrolase L1 (UCHL1), a member of the ubiquitin proteasome pathway, was consistently up-regulated in smokers compared with nonsmokers...
  42. ncbi Reversible monoubiquitination regulates the Parkinson disease-associated ubiquitin hydrolase UCH-L1
    Robin K Meray
    Department of Neurology, Harvard Medical School and Brigham and Women s Hospital, Cambridge, Massachusetts 02139, USA
    J Biol Chem 282:10567-75. 2007
    ..Our results illustrate monoubiquitination as a reversible regulatory mechanism for DUB activity involving auto-deubiquitination...
  43. ncbi Ubiquitin carboxyl-terminal hydrolase L1, a novel deubiquitinating enzyme in the vasculature, attenuates NF-kappaB activation
    Yoichi Takami
    Department of Geriatric Medicine, Osaka University Graduate School of Medicine, 2 2 Yamada oka, Suita 565 0871, Japan
    Arterioscler Thromb Vasc Biol 27:2184-90. 2007
    We identified a ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) gene, which encodes a deubiquitinating enzyme and is expressed in the vasculature, by functional screening of a human endothelial cell (EC) cDNA library...
  44. doi Aberrant molecular properties shared by familial Parkinson's disease-associated mutant UCH-L1 and carbonyl-modified UCH-L1
    Tomohiro Kabuta
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo 187 8502, Japan
    Hum Mol Genet 17:1482-96. 2008
    ..Our results also suggest that the carbonyl modification of UCH-L1 and subsequent abnormal interactions of carbonyl-modified UCH-L1 with multiple proteins, including tubulin, constitute one of the causes of sporadic PD...
  45. doi The S18Y polymorphic variant of UCH-L1 confers an antioxidant function to neuronal cells
    Elli Kyratzi
    Division of Basic Neurosciences, Biomedical Research Foundation, Academy of Athens, Athens, Greece
    Hum Mol Genet 17:2160-71. 2008
    ..Our results furthermore provide indirect evidence for the importance of oxidative stress as a pathogenetic factor in certain forms of sporadic PD...
  46. doi CpG hypermethylation of the UCHL1 gene promoter is associated with pathogenesis and poor prognosis in renal cell carcinoma
    Ichiro Kagara
    Department of Urology, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
    J Urol 180:343-51. 2008
    ..We performed microarray analysis in the renal cancer cell line ACHN treated with the demethylating agent 5-aza-2'-deoxycytidine and investigated the UCHL1 gene involved in the regulation of cellular ubiquitin levels.
  47. pmc The role of PGP9.5 as a tumor suppressor gene in human cancer
    Yutaka Tokumaru
    Department of Otolaryngology, Head and Neck Cancer Research Institute, Johns Hopkins University, Baltimore, MD, USA
    Int J Cancer 123:753-9. 2008
    ..Our data support the notion that PGP9.5 is a tumor suppressor gene that is inactivated by promoter methylation or gene deletion in several types of human cancers...
  48. doi Epigenetic identification of ubiquitin carboxyl-terminal hydrolase L1 as a functional tumor suppressor and biomarker for hepatocellular carcinoma and other digestive tumors
    Jun Yu
    Department of Medicine, Institute of Digestive Disease, Chinese University of Hong Kong, Hong Kong
    Hepatology 48:508-18. 2008
    The ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) is a carboxyl-terminal ubiquitin hydrolase regulating cellular ubiquitin levels, recently suggested as a tumor suppressor...
  49. doi Ubiquitin C-terminal hydrolase-L1 is a key regulator of tumor cell invasion and metastasis
    H J Kim
    The Center for Cell Signaling and Drug Discovery Research, College of Pharmacy and Division of Life and Pharmaceutical Sciences, Ewha Womans University, Seoul, Republic of Korea
    Oncogene 28:117-27. 2009
    ..In Akt-negative mutants, overexpression of UCH-L1 does not affect the invasion and migration capability of H157 cells. These results suggest that UCH-L1 is a key molecule to regulate tumor-cell invasion by upstream activation of Akt...
  50. pmc Over-expression of ubiquitin carboxy terminal hydrolase-L1 induces apoptosis in breast cancer cells
    Wen Juan Wang
    Department of Pathology, Shanghai Medical College, Fudan University, Shanghai 200032, PR China
    Int J Oncol 33:1037-45. 2008
    ..Our findings suggest that modulating the ubiquitination and deubiquitination pathway could be a novel method for tumor therapy...
  51. doi PGP 9.5 expression in cutaneous keratoacanthomas and squamous cell carcinomas
    Aikaterini Mastoraki
    4th Department of Surgery, Attikon University Hospital, Medical School, Athens University, 12462 Chaidari, Athens, Greece
    Arch Dermatol Res 301:653-8. 2009
    ..PGP 9.5 expression proved closely associated with tumor aggressiveness and is classified as a marker for predicting the outcome of resection-treated skin cancer patients...
  52. pmc Positive reciprocal regulation of ubiquitin C-terminal hydrolase L1 and beta-catenin/TCF signaling
    Anjali Bheda
    Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, United States of America
    PLoS ONE 4:e5955. 2009
    ..Ubiquitin C-terminal Hydrolase L1 (UCH L1) has been linked to several neurological diseases as well as human cancer, but the physiological targets and the ..
  53. doi Polymorphism of HD and UCHL-1 genes in Huntington's disease
    Er he Xu
    Department of Neurology, Xuanwu Hospital, Capital University of Medical Sciences, Beijing 100053, China
    J Clin Neurosci 16:1473-7. 2009
    ..UCHL-1 S18Y polymorphism is a modifier of HD with a modest regulatory role in the age at disease onset, suggesting that UCHL-1 may be involved in HD pathogenesis...
  54. pmc Expression and functional studies of ubiquitin C-terminal hydrolase L1 regulated genes
    Anjali Bheda
    Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, North Carolina, USA
    PLoS ONE 4:e6764. 2009
    ..One member of the UCH family, UCH L1, is expressed in a number of malignancies suggesting that this DUB might be involved in oncogenic processes, and ..
  55. doi Genomic screening for genes upregulated by demethylation revealed novel targets of epigenetic silencing in breast cancer
    Tomoko Fujikane
    Department of Molecular Biology, Cancer Research Institute, Sapporo Medical University, Sapporo 060 8556, Japan
    Breast Cancer Res Treat 122:699-710. 2010
    ..Thus, DNA methylation appears to be a common event in breast cancer, and the genes silenced by methylation could be useful targets for both diagnosis and therapy...
  56. pmc Association between the ubiquitin carboxyl-terminal esterase L1 gene (UCHL1) S18Y variant and Parkinson's Disease: a HuGE review and meta-analysis
    Margaret Ragland
    Department of Epidemiology, University of Washington, Center for Genomics and Public Health, Box 354921, 6200 NE 74th Street, Building 29, Suite 250, Seattle, WA 98115, USA
    Am J Epidemiol 170:1344-57. 2009
    The ubiquitin carboxyl-terminal esterase L1 gene, UCHL1, located on chromosome 4p14, has been studied as a potential candidate gene for Parkinson's disease risk...
  57. pmc Cyclopentenone prostaglandin-induced unfolding and aggregation of the Parkinson disease-associated UCH-L1
    Leonardus M I Koharudin
    Department of Structural Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15260, USA
    Proc Natl Acad Sci U S A 107:6835-40. 2010
    ..Our findings suggest a possible mechanistic link between UCH-L1 modification by cyclopentenone prostaglandins and the etiology of neurodegeneration...
  58. pmc Ubiquitin carboxy-terminal hydrolase L1 (UCHL1) S18Y polymorphism in Alzheimer's disease
    Madeleine Zetterberg
    Institute of Neuroscience and Physiology, Department of Clinical Neuroscience and Rehabilitation, Section of Ophthalmology, The Sahlgrenska Academy at the University of Gothenburg, Molndal, Sweden
    Mol Neurodegener 5:11. 2010
    ..Ubiquitin carboxy-terminal hydrolase L1 (UCHL1) is a de-ubiquitinating enzyme with important functions in recycling of ubiquitin...
  59. doi Ubiquitin specific proteases USP24 and USP40 and ubiquitin thiolesterase UCHL1 polymorphisms have synergic effect on the risk of Parkinson's disease among Taiwanese
    Yih Ru Wu
    Department of Neurology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taipei, Taiwan
    Clin Chim Acta 411:955-8. 2010
    ..Impaired ubiquitin-proteasome system function may contribute to the pathogenesis of Parkinson's disease (PD)...
  60. pmc Ubiquitin vinyl methyl ester binding orients the misaligned active site of the ubiquitin hydrolase UCHL1 into productive conformation
    David A Boudreaux
    Department of Chemistry, Purdue University, 560 Oval Drive, West Lafayette, IN 47907, USA
    Proc Natl Acad Sci U S A 107:9117-22. 2010
    Ubiquitin carboxy-terminal hydrolase L1 (UCHL1) is a Parkinson disease-associated, putative cysteine protease found abundantly and selectively expressed in neurons...
  61. pmc The de-ubiquitinase UCH-L1 is an oncogene that drives the development of lymphoma in vivo by deregulating PHLPP1 and Akt signaling
    S Hussain
    Department of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, MN 55906, USA
    Leukemia 24:1641-55. 2010
    ..These data provide the first in vivo evidence for DUB-driven oncogenesis and suggest that UCH-L1 hyperactivity deregulates normal Akt signaling...
  62. doi The effect of Parkinson's-disease-associated mutations on the deubiquitinating enzyme UCH-L1
    Fredrik I Andersson
    Department of Chemistry, University of Cambridge, Cambridge, UK
    J Mol Biol 407:261-72. 2011
    ....
  63. pmc Biokinetic analysis of ubiquitin C-terminal hydrolase-L1 (UCH-L1) in severe traumatic brain injury patient biofluids
    Gretchen M Brophy
    Virginia Commonwealth University, Pharmacotherapy and Outcomes Sciences and Neurosurgery, Richmond, Virginia 23298 0533, USA
    J Neurotrauma 28:861-70. 2011
    ..This study shows a significant correlation between UCH-L1 CSF and serum median concentrations and biokinetics in severe TBI patients, and relationships with clinical outcome were detected...
  64. pmc The UCHL1 S18Y polymorphism and Parkinson's disease in a Japanese population
    Katherine W Snapinn
    Department of Epidemiology, University of Washington School of Public Health, Seattle, Washington, USA
    Parkinsonism Relat Disord 17:473-5. 2011
    b>UCHL1 plays an important role in the ubiquitin-proteasome system and is a biologically plausible candidate gene for Parkinson's disease (PD)...
  65. doi Elevated serum ubiquitin carboxy-terminal hydrolase L1 is associated with abnormal blood-brain barrier function after traumatic brain injury
    Brian J Blyth
    Department of Emergency Medicine and Center for Neural Development and Disease, University of Rochester Medical Center, Rochester, New York 14642, USA
    J Neurotrauma 28:2453-62. 2011
    ..Ubiquitin C-terminal hydrolase 1 (UCHL1), and phosphorylated neurofilament heavy chain (pNF-H) are found exclusively in neurons, but their relationship to ..
  66. doi The serine protease HtrA2 cleaves UCH-L1 and inhibits its hydrolase activity: implication in the UCH-L1-mediated cell death
    Dae Wook Park
    Department of Biomedical Sciences, College of Medicine, The Catholic University of Korea, Seoul 137 701, Republic of Korea
    Biochem Biophys Res Commun 415:24-9. 2011
    ..These findings suggest that the HtrA2-mediated cleavage of UCH-L1 may play important roles in regulating the fine balance between cell growth and cell death...
  67. doi Frequent CpG methylation of ubiquitin carboxyl-terminal hydrolase 1 (UCHL1) in sporadic and hereditary Tunisian breast cancer patients: clinical significance
    Fatma Trifa
    Laboratory of Biomass Valorisation and Production of Eukaryotic Proteins, Center of Biotechnology of Sfax, University of Sfax, Sidi Mansour Street, BP K 1177, 3018 Sfax, Tunisia
    Med Oncol 30:418. 2013
    ..In this study, we aimed to assess, by methylation-specific PCR, the CpG methylation pattern of the UCHL1 promoter in 94 sporadic and 44 hereditary breast cancers from Tunisian patients...
  68. pmc Recessive loss of function of the neuronal ubiquitin hydrolase UCHL1 leads to early-onset progressive neurodegeneration
    Kaya Bilguvar
    Department of Neurosurgery, Yale School of Medicine, New Haven, CT 06510, USA
    Proc Natl Acad Sci U S A 110:3489-94. 2013
    Ubiquitin C-terminal hydrolase-L1 (UCHL1), a neuron-specific de-ubiquitinating enzyme, is one of the most abundant proteins in the brain...
  69. pmc Human papillomavirus (HPV) upregulates the cellular deubiquitinase UCHL1 to suppress the keratinocyte's innate immune response
    Rezaul Karim
    Center for Human and Clinical Genetics, Leiden University Medical Center, Leiden, The Netherlands
    PLoS Pathog 9:e1003384. 2013
    ..was shown to depend on hrHPV-induced expression of the cellular protein ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) in keratinocytes...
  70. ncbi An Ile93Met substitution in the UCH-L1 gene is not a disease-causing mutation for idiopathic Parkinson's disease
    Qiaoyun Shi
    Department of Internal Medicine, Second Affiliated Hospital, Sun Yat Sen Memorial Hospital, Sun Yat Sen University, Guangzhou 510120, China
    Chin Med J (Engl) 116:312-3. 2003
    ..To ascertain whether a coding mutation (Ile93Met) in ubiquitin carboxy-terminal hydrolase (UCH-L1) gene plays a role in idiopathic Parkinson's disease (IPD)...
  71. ncbi Complex interactions in Parkinson's disease: a two-phased approach
    Demetrius M Maraganore
    Department of Neurology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA
    Mov Disord 18:631-6. 2003
    ..of pathogenic mutations in the three genes alpha-synuclein, parkin, and ubiquitin carboxy-terminal hydrolase L1 (UCHL1) has elucidated the ubiquitin proteasome system (UPS) and its potential role as a causal pathway in Parkinson's ..
  72. ncbi Genetic causes of Parkinson's disease: UCHL-1
    Daniel G Healy
    Department of Molecular Neuroscience, Institute of Neurology, Queen Square, WC1N 3BG, London, UK
    Cell Tissue Res 318:189-94. 2004
    ..Important issues regarding UCHL-1 and its role in PD remain inconclusive, especially regarding the pathogenicity of the mendelian I93M mutation. This review tries to address some of these uncertainties...
  73. ncbi UCH-L1 aggresome formation in response to proteasome impairment indicates a role in inclusion formation in Parkinson's disease
    Helen C Ardley
    Molecular Medicine Unit, University of Leeds, Clinical Sciences Building, St James s University Hospital, Leeds, UK
    J Neurochem 90:379-91. 2004
    ..Furthermore, UCH-L1 accumulation is likely to play a pathological role in inclusion formation in Parkinson's disease...
  74. ncbi Activity profiling of deubiquitinating enzymes in cervical carcinoma biopsies and cell lines
    Ulrika Rolen
    Microbiology and Tumor Biology Center, Karolinska Institutet, Stockholm, Sweden
    Mol Carcinog 45:260-9. 2006
    ..UCH-L1, UCH-L3, USP7, and USP9X activity was upregulated following HPV E6/E7 immortalization of keratinocytes, suggesting a role of these enzymes in growth transformation...
  75. ncbi Analysis of functional polymorphisms in three synaptic plasticity-related genes (BDNF, COMT AND UCHL1) in Alzheimer's disease in Colombia
    Diego A Forero
    Grupo de Neurociencias, Facultad de Medicina e Instituto de Genética, Universidad Nacional de Colombia, Bogota, Colombia
    Neurosci Res 55:334-41. 2006
    ..factor, BDNF Val66Met; catechol-O-methyl transferase, COMT Val158; ubiquitin carboxyl-terminal hydroxylase, UCHL1 S18Y) in a sample of 102 AD cases and 168 age and sex matched controls living in Bogotá, Colombia...
  76. ncbi Case-control study of UCHL1 S18Y variant in Parkinson's disease
    Eng King Tan
    Department of Neurology, Singapore General Hospital, Singapore
    Mov Disord 21:1765-8. 2006
    ..meta-analysis observed a greater significant inverse association of the ubiquitin carboxy-terminal hydrolase L1 (UCHL1) S18Y variant with Parkinson's disease (PD) for Asian (predominantly Japanese) populations compared with Caucasian ..
  77. ncbi S18Y in ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) associated with decreased risk of Parkinson's disease in Sweden
    Andrea Carmine Belin
    Department of Neuroscience, Karolinska Institutet, Retzius väg 8 B2 4, 171 77 Stockholm, Sweden
    Parkinsonism Relat Disord 13:295-8. 2007
    ..The tyrosine variant was significantly inversely associated with PD (P=0.049) and with a low age of onset (50 years) (P=0.017) in the case-control material, supporting the hypothesis of a protective function...
  78. doi Lack of evidence for association of a UCH-L1 S18Y polymorphism with Parkinson's disease in a Han-Chinese population
    Z J Zhang
    Department of Neurology, West China Hospital, Sichuan University, Chengdu 610041, Chengdu, China
    Neurosci Lett 442:200-2. 2008
    ..We did not observe any difference in allele or genotype frequencies between the cases and the controls (P>0.05). Our results do not support a role for this variant in sporadic PD...
  79. doi Effects of UCH-L1 on alpha-synuclein over-expression mouse model of Parkinson's disease
    Toru Yasuda
    Research Institute for Diseases of Old Age, Juntendo University, Tokyo, Japan
    J Neurochem 108:932-44. 2009
    The rare inherited form of Parkinson's disease (PD), PARK5, is caused by a missense mutation in ubiquitin carboxy-terminal hydrolase-L1 (UCH-L1) gene, resulting in Ile93Met substitution in its gene product (UCH-L1(Ile93Met))...
  80. doi The tumor suppressor UCHL1 forms a complex with p53/MDM2/ARF to promote p53 signaling and is frequently silenced in nasopharyngeal carcinoma
    Lili Li
    State Key Laboratory in Oncology in South China Cancer Epigenetics Laboratory, Department of Clinical Oncology, Sir YK Pao Center for Cancer, Hong Kong Cancer Institute, Hong Kong
    Clin Cancer Res 16:2949-58. 2010
    ..This study investigated the role of ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) in nasopharyngeal carcinoma pathogenesis.
  81. doi Case-control study of the UCH-L1 S18Y variant in sporadic Parkinson's disease in the Chinese population
    Lei Wang
    Department of Neurology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, Hunan 410008, China
    J Clin Neurosci 18:541-4. 2011
    ....
  82. ncbi The ubiquitin carboxy-terminal hydrolase-L1 gene S18Y polymorphism does not confer protection against idiopathic Parkinson's disease
    G D Mellick
    Department of Medicine, University of Queensland, Princess Alexandra Hospital, Queensland, 4102, Woolloongabba, Australia
    Neurosci Lett 293:127-30. 2000
    ..The frequency of the S18Y allele in Australian subjects is similar to that reported elsewhere. This study suggests that the S18Y polymorphism in UCH-L1 does not influence the risk for developing PD...
  83. ncbi Lack of association between ubiquitin carboxy-terminal hydrolase L1 gene polymorphism and PD
    G Savettieri
    Institute of Neurology, University of Palermo, Italy
    Neurology 57:560-1. 2001
  84. ncbi No genetic association of the ubiquitin carboxy-terminal hydrolase-L1 gene S18Y polymorphism with familial Parkinson's disease
    C Levecque
    INSERM 508, Institut Pasteur de Lille, France
    J Neural Transm (Vienna) 108:979-84. 2001
    ..This observation suggests that the previous positive results obtained may reflect mechanisms restricted to the sporadic form of the disease or to a founder effect of the disease susceptibility...
  85. ncbi Identification of methylation-silenced genes in colorectal cancer cell lines: genomic screening using oligonucleotide arrays
    Satoshi Fukutomi
    Department of General Surgery, Chiba University Graduate School of Medicine, Chuoh ku, Chiba, Japan
    Scand J Gastroenterol 42:1486-94. 2007
    ..Aberrant methylation of promoter CpG islands is associated with the loss of expression of tumor suppressor genes in human cancers. The purpose of this study was to examine methylation-silenced genes in colorectal cancer (CRC) cell lines...
  86. ncbi UCHL1 is associated with Parkinson's disease: a case-unaffected sibling and case-unrelated control study
    Maurizio Facheris
    Department of Neurology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA
    Neurosci Lett 381:131-4. 2005
    ..disequilibrium test to confirm the association of the S18Y variant of the ubiquitin carboxi-terminal hydrolase L1 (UCHL1) gene with Parkinson's disease (PD)...
  87. ncbi Metastasizing neuroblastomas from taste buds in rats transgenic for the Simian virus 40 large T antigen under control of the probasin gene promoter
    M Asamoto
    First Department of Pathology, Nagoya City University Medical School, Nagoya, Japan
    Toxicol Pathol 29:363-8. 2001
    ..Castration or supplementation with testosterone propionate did not alter tumor development, indicating the tumors to be androgen-independent. These results clearly show that taste buds can give rise to metastasizing neuroblastomas...
  88. ncbi [Frontier researches for the development of molecular-targeted therapies for familial Parkinson disease]
    Yuzuru Imai
    Institute of Development, Aging and Cancer, Tohoku University, 4 1 Seiryo machi, Aoba ku, Sendai, Miyagi 980 8575, Japan
    Brain Nerve 61:903-13. 2009
    ....
  89. ncbi Mechanism of extracellular ubiquitination in the mammalian epididymis
    Kathleen M Baska
    Division of Animal Sciences, University of Missouri Columbia, Columbia, Missouri, USA
    J Cell Physiol 215:684-96. 2008
    ..5/UCHL1 in the isolated bovine EF...
  90. ncbi Differential expression of genes encoding constitutive and inducible 20S proteasomal core subunits in the testis and epididymis of theophylline- or 1,3-dinitrobenzene-exposed rats
    Mark W Tengowski
    Pfizer Global Research and Development, Ann Arbor, Michigan 48105, USA
    Biol Reprod 76:149-63. 2007
    ..and Psmb10 (LMP10); and Ube1 (ubiquitin-activating enzyme E1), Ube2d3 (ubiquitin-conjugating enzyme E2), and Uchl1 (ubiquitin C-terminal hydrolase PGP9.5)...
  91. ncbi Ubiquitin C-terminal hydrolase L-1 is essential for the early apoptotic wave of germinal cells and for sperm quality control during spermatogenesis
    Jungkee Kwon
    Department of Degenerative Neurological Disease, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
    Biol Reprod 73:29-35. 2005
    ..Previously, we found that UCHL1-deficient testes of gad mice have reduced ubiquitin levels and are resistant to cryptorchid stress-related injury...
  92. doi UCH-L1 expression of podocytes in diseased glomeruli and in vitro
    Yuan Liu
    Department of Pathology and Key Laboratory of Molecular Medicine, Ministry of Education, Shanghai Medical College, Fudan University, Shanghai 200032, People s Republic of China
    J Pathol 217:642-53. 2009
    ..The immune injury is a main cause for stimulating podocytes to express UCH-L1. The expression of UCH-L1 may be associated with the regeneration of podocytes as a repair response to immunocomplex-mediated injury...
  93. ncbi The region-specific functions of two ubiquitin C-terminal hydrolase isozymes along the epididymis
    Jungkee Kwon
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
    Exp Anim 55:35-43. 2006
    ..In conclusion, the response of gad and Uchl3 knockout mice to androgen withdrawal suggests a reciprocal function of the two UCH enzymes in the caput epididymis...
  94. ncbi Developmental regulation of ubiquitin C-terminal hydrolase isozyme expression during spermatogenesis in mice
    Jungkee Kwon
    Department of Biomedical Science, Graduate School of Agricultural and Life Sciences, University of Tokyo, 1 1 1 Yayoi, Bunkyo ku, Tokyo 113 8657, Japan
    Biol Reprod 71:515-21. 2004
    ....
  95. ncbi Neuron-specific PGP9.5 expression in rat hair follicle development and cycle
    T Ohsawa
    Department of Bioactivity Regulation, Metropolitan Institute of Gerontology of Tokyo, 35 2 Sakae cho, Itabashi ku, 173 0015, Tokyo, Japan
    J Dermatol Sci 26:100-5. 2001
    ..These findings showed that PGP9.5 is not specific to the neuron but is also involved in the hair follicle, and should provide new insight into the development and regression of the hair follicle...
  96. ncbi Ubiquitin C-terminal hydrolase-activity is involved in sperm acrosomal function and anti-polyspermy defense during porcine fertilization
    Young Joo Yi
    Department of Anatomy, University of Missouri Columbia, Missouri 65211, USA
    Biol Reprod 77:780-93. 2007
    ..Antibodies against two closely related UCHs, UCHL1 and UCHL3, detected these UCHs in the oocyte cortex and on the sperm acrosome, respectively, and increased the ..
  97. doi Role of ubiquitin C-terminal hydrolase-L1 in antipolyspermy defense of mammalian oocytes
    Andrej Susor
    Institute of Animal Physiology and Genetics, Academy of Sciences of the Czech Republic, Libechov, Czech Republic
    Biol Reprod 82:1151-61. 2010
    ..Ubiquitin C-terminal hydrolase-L1 (UCHL1) is one of the most abundant proteins in mammalian oocytes...
  98. doi Deficiency of ubiquitin carboxy-terminal hydrolase-L1 (UCH-L1) leads to vulnerability to lipid peroxidation
    Satoshi Nagamine
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo 187 8502, Japan
    Neurochem Int 57:102-10. 2010
    ..These findings will provide clues for elucidating the physiological function of UCH-L1...
  99. ncbi Loss of Uch-L1 and Uch-L3 leads to neurodegeneration, posterior paralysis and dysphagia
    L J Kurihara
    Howard Hughes Medical Institute and Department of Molecular Biology, Princeton University, Princeton, NJ 08544, USA
    Hum Mol Genet 10:1963-70. 2001
    ..This study is the first to successfully document dysphagia in the mouse and is a potentially valuable resource for understanding human neurodegenerative disorders that cause swallowing defects...
  100. ncbi A role of the ubiquitin-proteasome system in neuropathic pain
    Andrew Moss
    Department of Preclinical Veterinary Sciences, The Royal Dick School of Veterinary Studies, The University of Edinburgh, Summerhall, Edinburgh EH9 1QH, United Kingdom
    J Neurosci 22:1363-72. 2002
    ....
  101. ncbi Expression of protein gene product 9.5 (PGP9.5)/ubiquitin-C-terminal hydrolase 1 (UCHL-1) in human myeloma cells
    T Otsuki
    Department of Hygiene, Kawasaki Medical School, 577 Matsushima, Kurashiki, Japan
    Br J Haematol 127:292-8. 2004
    ..5. These results suggested that PGP9.5 may be a good marker for myeloma among haematological malignancies. In addition, it may indicate certain cellular features of myeloma cells, such as sensitivity to proteasome inhibitors...

Research Grants37

  1. Pembe Hande Ozdinler; Fiscal Year: 2016
    ..Ubiquitin carboxy-terminal hydrolase L1 (UCHL1) is a unique DUB with both ligase and hydrolase activities and it is gaining much attention after identification ..
  2. High Throughout Assay to Probe UCH-L1 Ligase Inhibitors
    Peter Lansbury; Fiscal Year: 2005
    ..A gene, PARK5, has been linked to PD are involved in proteasomal degradation pathway and it is an ubiquitin C terminal hydrolase ..
  3. Structure and Mechanism of the Parkin Ubiquitin Ligase
    Brenda Schulman; Fiscal Year: 2003
    ..The PARK5 gene is thought to encode UCH-L1, a ubiquitin C terminal hydrolase...
  4. Molecular Epidemiology of Parkinson's Disease
    DEMETRIUS MICHAEL MARAGANORE; Fiscal Year: 2010
    ..The linkage-derived genes alpha-synuclein (SNCA), ubiquitin carboxy-terminal hydrolase L1 (UCHL1), microtubule-associated protein tau (MAPT), and parkin (PRKN) confer PD susceptibility, possibly via their ..
  5. role of ubiquitinin in cancer progression
    Jin Ming Yang; Fiscal Year: 2005
    ..abstract_text> ..
  6. Conditional Uch-L1 knockout mice
    Michael Lee; Fiscal Year: 2005
    ..Specifically, we will generate UchL1-floxed mice to conditionally silence Uch-L1 expression by mating to appropriate Cre expressing mice...
  7. MOLECULAR MECHANISMS OF THE PARKINSON'S DISEASE GENES
    Asa Abeliovich; Fiscal Year: 2007
    ..Of particular interest are potential relationships among these molecules. I propose to combine complementary biochemical, cellular, and genetic approaches to this end. ..
  8. Amy L Wilson-Delfosse; Fiscal Year: 2014
    ..abstract_text> ..
  9. Role of UCH-L1 Oxidative Modification in Parkinson's Disease Pathogenesis
    JEANNE ELIZABETH MCKEON; Fiscal Year: 2012
    ....
  10. Steven H Graham; Fiscal Year: 2016
    ..The current project aims to identify a downstream mechanism by which COX2 activity injures neurons which could lead to new treatments for stroke and neurodegenerative diseases. ..
  11. Pembe Hande Ozdinler; Fiscal Year: 2014
    ..We recently generated a novel reporter line, the UCHL1-eGFP mice, in which CSMN are genetically labeled in the motor cortex...
  12. Integration of Immunochemical, Genomic & Transcriptomic Analyses of Human Skin
    FRANK LAMBERT contact RICE; Fiscal Year: 2010
    ..The results will greatly aid the development of novel and efficacious therapeutic interventions directed at the skin. ..
  13. Sheng He Huang; Fiscal Year: 2014
    ..NT and gp120 were able to significantly increase the serum levels of UCHL1 (a new BBB marker) as well as S100B in mice, which are correlated with the changes in cBMEC and EPC...
  14. Beate R Ritz; Fiscal Year: 2015
    ..3) Investigate novel gene-pesticide interactions for recognized PD risk genes (PARK1/PARK4 SNCA, PARK2 parkin, PARK5 UCHL1, PARK6 PINK, PARK8 LRRK2, and PARK13 HTRA2)...
  15. Biochemical Markers of Traumatic Brain Injury
    STEVEN ANDREW ROBICSEK; Fiscal Year: 2010
    ....
  16. MICHAEL ROSS FRANKEL; Fiscal Year: 2015
    ..We will also explore whether diminished release of S100b, GFAP, UCH-L1 and SBDP150, measured at 24 and 48 hours, can serve as a readily identifiable surrogate measure of early treatment response to progesterone. ..
  17. William M Valentine; Fiscal Year: 2014
    ..for environmental agents based upon familial forms of PD resulting from loss of function mutations in Parkin and UCHL1 that are both components of the ubiquitin pathway...
  18. Blossom A Damania; Fiscal Year: 2016
    ..Project 2 continues the study by Dr, Joseph Pagano on a novel deubiquitinase, UCHL1, whose expression is induced by EBV...
  19. PAUL JOHN GALARDY; Fiscal Year: 2015
    ..In the first aim, we will mate Uchl1 transgenic mice with models of biologically distinct B-cell malignancies to understand the contribution of UCH-L1 ..
  20. NEUROPLASTICITY OF THE AGING BLADDER
    Toby Chai; Fiscal Year: 2000
    ....
  21. STRUCTURAL STUDIES ON UBIQUITIN C-TERMINAL HYDROLASES
    Christopher Hill; Fiscal Year: 1999
    ..The amino acid sequence around the active site cysteine of Yuh1 has some similarity to the putative active site region of the human oncoprotein Tre-2, which is also a UCH. ..
  22. Production and characterization of ubiquitin proteins
    KIRSTY LAPAN; Fiscal Year: 2006
    ..unreadable] [unreadable]..
  23. Parkin, Ubiquitination and Cell Death
    Leonard Petrucelli; Fiscal Year: 2003
    ....
  24. FAMILIAL PARKINSON'S DISEASE: CLUES TO PATHOGENESIS
    Peter Lansbury; Fiscal Year: 2009
    ..Such molecules will be tested in animal models of disease. To enable the first step in such testing, a core facility for testing of drug-like compounds in Parkinsonian drosophila has been established (core B). ..
  25. HTS for Inhibitors of BAP1,BRCA:Deubiquinating(RMI)
    KEITH WILKINSON; Fiscal Year: 2005
    ..This screen will result in useful molecular probes of BAP1 function and help to clarify the role of BAP1 in BRCA1 mediated events. ..
  26. Neurite density in skin as a marker for neuropathic pain
    Anne Louise Oaklander; Fiscal Year: 2005
    ..abstract_text> ..
  27. Developing Biomarker-Based Prognostics In Breast Cancer
    John Olson; Fiscal Year: 2008
    ..abstract_text> ..
  28. Fetal Lung Development:Role of Wnt5a Signaling
    Changgong Li; Fiscal Year: 2008
    ..This information will be of utility in understanding the molecular mechanisms of congenital and induced lung disease. ..
  29. ESCHERICHIA COLI INVASION OF BRAIN ENDOTHELIAL CELLS
    Sheng He Huang; Fiscal Year: 2007
    ..To further characterize the role of IbeABP in E. coil K1 invasion of human BMEC. (3). To determine how E. coil K1 invasion gene expression is regulated by the IbeR and environmental signals (glucose and oxygen tension). ..
  30. Biomarker Genes in Mood Disorder: Lymphocyte and Brain
    MARQUIS VAWTER; Fiscal Year: 2006
    ..The potential use of biomarkers in lymphocytes will provide clinical researchers a tool to better address questions concerning diagnostic subgroups and treatment responders and non-responders. ..
  31. Molecular analysis of DJ-1 function and dysfunction
    Lih Shen Chin; Fiscal Year: 2010
    ..Completion of the proposed project should advance our understanding of PD pathogenesis, and accelerate the efforts to discover curative therapies for PD and related neurodegenerative disorders. ..
  32. Improving genomic prediction models in breast cancer
    John Olson; Fiscal Year: 2005
    ....
  33. Uterus & Colon Influences on Bladder Function in Females
    Karen Berkley; Fiscal Year: 2004
    ..Results from these exploratory studies will be then be used to develop a longer program of research. ..
  34. Surrogate markers for brain damage
    Robert Siman; Fiscal Year: 2009
    ....
  35. Biophysical Characterization of Alpha-Synuclein
    Ellen Doss Pepe; Fiscal Year: 2004
    ..The long-term goal of this aim is to provide a mechanism for the formation of Lewy bodies. ..
  36. Using Biochemical Markers to Detect Abusive Head Trauma
    Rachel Berger; Fiscal Year: 2007
    ..abstract_text> ..