WTX

Summary

Gene Symbol: WTX
Description: APC membrane recruitment protein 1
Alias: FAM123B, OSCS, WTX, Wilms tumor gene on the X chromosome protein, Wilms tumor on the X, adenomatous polyposis coli membrane recruitment 1, family with sequence similarity 123B, protein FAM123B
Species: human

Top Publications

  1. ncbi Wilms tumor suppressor WTX negatively regulates WNT/beta-catenin signaling
    Michael B Major
    Howard Hughes Medical Institute, University of Washington School of Medicine, Box 357370, Seattle, WA 98195, USA
    Science 316:1043-6. 2007
  2. doi Osteopathia striata with cranial sclerosis owing to WTX gene defect
    Bram Perdu
    Department of Medical Genetics, University and University Hospital of Antwerp, 2610 Antwerp, Belgium
    J Bone Miner Res 25:82-90. 2010
  3. pmc Amer1/WTX couples Wnt-induced formation of PtdIns(4,5)P2 to LRP6 phosphorylation
    Kristina Tanneberger
    Nikolaus Fiebiger Center, University Erlangen Nurnberg, Erlangen, Germany
    EMBO J 30:1433-43. 2011
  4. doi Germline mutations in WTX cause a sclerosing skeletal dysplasia but do not predispose to tumorigenesis
    Zandra A Jenkins
    Departments of Paediatrics, Dunedin School of Medicine, Otago University, Dunedin 9054, New Zealand
    Nat Genet 41:95-100. 2009
  5. doi Functional inactivation of the WTX gene is not a frequent event in Wilms' tumors
    D Perotti
    Department of Experimental Oncology and Laboratories, Genetic Susceptibility to Cancer Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
    Oncogene 27:4625-32. 2008
  6. doi WTX is rarely mutated in acute myeloid leukemia
    Carolyn Owen
    Haematologica 93:947-8. 2008
  7. ncbi Osteopathia striata cranial sclerosis: non-random X-inactivation suggestive of X-linked dominant inheritance
    Geraldine Viot
    Department of Genetics, Hopital Necker Enfants Malades, Paris, France
    Am J Med Genet 107:1-4. 2002
  8. doi Functional characterization of Wilms tumor-suppressor WTX and tumor-associated mutants
    M K H Kim
    Division of Hematology Oncology, Department of Medicine, Feinberg School of Medicine, Robert H Lurie Comprehensive Cancer Center, Northwestern University, Chicago, IL, USA
    Oncogene 30:832-42. 2011
  9. doi WTX mutations can occur both early and late in the pathogenesis of Wilms tumour
    Ryuji Fukuzawa
    Department of Women s and Children s Health, Dunedin School of Medicine, University of Otago, PO Box 913 Dunedin, New Zealand
    J Med Genet 47:791-4. 2010
  10. pmc The tumor suppressor WTX shuttles to the nucleus and modulates WT1 activity
    Miguel N Rivera
    Massachusetts General Hospital Cancer Center, Harvard Medical School, Charlestown, MA 20129, USA
    Proc Natl Acad Sci U S A 106:8338-43. 2009

Scientific Experts

Detail Information

Publications75

  1. ncbi Wilms tumor suppressor WTX negatively regulates WNT/beta-catenin signaling
    Michael B Major
    Howard Hughes Medical Institute, University of Washington School of Medicine, Box 357370, Seattle, WA 98195, USA
    Science 316:1043-6. 2007
    ..This assay revealed that WTX, a protein encoded by a gene mutated in Wilms tumors, forms a complex with beta-catenin, AXIN1, beta-TrCP2 (beta-..
  2. doi Osteopathia striata with cranial sclerosis owing to WTX gene defect
    Bram Perdu
    Department of Medical Genetics, University and University Hospital of Antwerp, 2610 Antwerp, Belgium
    J Bone Miner Res 25:82-90. 2010
    ..Recently, the disease-causing gene was identified as the WTX gene (FAM123B), an inhibitor of WNT signaling...
  3. pmc Amer1/WTX couples Wnt-induced formation of PtdIns(4,5)P2 to LRP6 phosphorylation
    Kristina Tanneberger
    Nikolaus Fiebiger Center, University Erlangen Nurnberg, Erlangen, Germany
    EMBO J 30:1433-43. 2011
    ..Here, we show that adenomatous polyposis coli membrane recruitment 1 (Amer1) (also called WTX), a membrane associated PtdIns(4,5)P(2)-binding protein, is ..
  4. doi Germline mutations in WTX cause a sclerosing skeletal dysplasia but do not predispose to tumorigenesis
    Zandra A Jenkins
    Departments of Paediatrics, Dunedin School of Medicine, Otago University, Dunedin 9054, New Zealand
    Nat Genet 41:95-100. 2009
    ..Here we demonstrate that germline mutations in WTX (FAM123B), a gene that encodes a repressor of canonical WNT signaling, cause an X-linked sclerosing bone dysplasia, ..
  5. doi Functional inactivation of the WTX gene is not a frequent event in Wilms' tumors
    D Perotti
    Department of Experimental Oncology and Laboratories, Genetic Susceptibility to Cancer Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
    Oncogene 27:4625-32. 2008
    For many years the precise genetic etiology of the majority of Wilms' tumors has remained unexplained. Recently, the WTX gene, mapped to chromosome Xq11...
  6. doi WTX is rarely mutated in acute myeloid leukemia
    Carolyn Owen
    Haematologica 93:947-8. 2008
  7. ncbi Osteopathia striata cranial sclerosis: non-random X-inactivation suggestive of X-linked dominant inheritance
    Geraldine Viot
    Department of Genetics, Hopital Necker Enfants Malades, Paris, France
    Am J Med Genet 107:1-4. 2002
    ..This finding, in combination with a sex ratio in favor of females and an increased morbidity and mortality in males, is highly suggestive of X-linked dominant inheritance...
  8. doi Functional characterization of Wilms tumor-suppressor WTX and tumor-associated mutants
    M K H Kim
    Division of Hematology Oncology, Department of Medicine, Feinberg School of Medicine, Robert H Lurie Comprehensive Cancer Center, Northwestern University, Chicago, IL, USA
    Oncogene 30:832-42. 2011
    The WTX, Wilms tumor-associated tumor-suppressor gene, is present on the X chromosome and a single WTX mutation may be sufficient to promote carcinogenesis...
  9. doi WTX mutations can occur both early and late in the pathogenesis of Wilms tumour
    Ryuji Fukuzawa
    Department of Women s and Children s Health, Dunedin School of Medicine, University of Otago, PO Box 913 Dunedin, New Zealand
    J Med Genet 47:791-4. 2010
    ..in the same gene cause the sclerosing skeletal dysplasia, osteopathia striata congenita with cranial sclerosis (OSCS)...
  10. pmc The tumor suppressor WTX shuttles to the nucleus and modulates WT1 activity
    Miguel N Rivera
    Massachusetts General Hospital Cancer Center, Harvard Medical School, Charlestown, MA 20129, USA
    Proc Natl Acad Sci U S A 106:8338-43. 2009
    b>WTX encodes a tumor suppressor gene inactivated in Wilms tumor and recently implicated in WNT signaling through enhancement of cytoplasmic beta-catenin (CTNNB1) degradation...
  11. doi Wilms tumor genetics: mutations in WT1, WTX, and CTNNB1 account for only about one-third of tumors
    E Cristy Ruteshouser
    Department of Cancer Genetics, The University of Texas M D Anderson Cancer Center, Houston, TX 77030, USA
    Genes Chromosomes Cancer 47:461-70. 2008
    ..However, WT1 is altered in only approximately 20% of Wilms tumors. Recently a novel gene, WTX at Xq11.1, was reported to be mutated in Wilms tumors...
  12. doi Mutational analysis of WTX gene in Wnt/ beta-catenin pathway in gastric, colorectal, and hepatocellular carcinomas
    Nam J Yoo
    Department of Pathology, College of Medicine, The Catholic University of Korea, 505 Banpo Dong, Socho gu, Seoul 137 701, Korea
    Dig Dis Sci 54:1011-4. 2009
    A recent study of Wilms' tumors discovered a new X chromosome gene, Wilms' tumor gene on the X chromosome (WTX), which was found to harbor small deletions and point mutations...
  13. ncbi AMER1 regulates the distribution of the tumor suppressor APC between microtubules and the plasma membrane
    Annette Grohmann
    Nikolaus Fiebiger Center for Molecular Medicine, University Erlangen Nuremberg, Glückstr 6, 91054 Erlangen, Germany
    J Cell Sci 120:3738-47. 2007
    ..These data indicate that AMER1 controls the subcellular distribution of APC between membrane- and microtubule-associated pools, and might thereby regulate APC-dependent cellular morphogenesis, cell migration and cell-cell adhesion...
  14. ncbi An X chromosome gene, WTX, is commonly inactivated in Wilms tumor
    Miguel N Rivera
    Massachusetts General Hospital Cancer Center, Harvard Medical Center, Boston, MA 02114, USA
    Science 315:642-5. 2007
    ..This gene, which we call WTX, is inactivated in approximately one-third of Wilms tumors (15 of 51 tumors)...
  15. ncbi Promoter methylation of Wilms' tumor gene on the X- chromosome in gastric cancer
    Xia Liu
    Department of Pathology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China
    Nan Fang Yi Ke Da Xue Xue Bao 33:318-21. 2013
    To investigate the changes in methylation levels of the promoters of the tumor suppressor gene Wilms' tumor gene on the X-chromosome (WTX) and its possible role in gastric cancer.
  16. doi Osteopathia striata congenita with cranial sclerosis and intellectual disability due to contiguous gene deletions involving the WTX locus
    S K Holman
    Department of Paediatrics, Dunedin School of Medicine, Otago University, Dunedin, New Zealand
    Clin Genet 83:251-6. 2013
    ..is a skeletal dysplasia caused by germline deletions of or truncating point mutations in the X-linked gene WTX (FAM123B, AMER1)...
  17. pmc Gain of FAM123B and ARHGEF9 in an Obese Man with Intellectual Disability, Congenital Heart Defects and Multiple Supernumerary Ring Chromosomes
    R Hochstenbach
    Department of Medical Genetics, University Medical Centre Utrecht, Utrecht, The Netherlands
    Mol Syndromol 3:274-83. 2013
    ..The r(X) was present in 84-89% of cells in the various tissues examined, lacks the XIST gene, but contains FAM123B, a potential dosage-sensitive candidate gene for congenital cardiac abnormalities, and ARHGEF9, a candidate gene ..
  18. doi Two novel WTX mutations underscore the unpredictability of male survival in osteopathia striata with cranial sclerosis
    B Perdu
    Department of Medical Genetics, University Hospital of Antwerp, University of Antwerp, Edegem, Belgium
    Clin Genet 80:383-8. 2011
    ..Recently, the disease-causing gene was identified as the WTX gene (FAM123B)...
  19. doi Defects in the DNA mismatch repair system do not contribute to the development of childhood wilms tumors
    Heidi Segers
    Department of Pediatric Oncology Hematology, Erasmus MC Sophia Children s Hospital, Dr Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands
    Pediatr Dev Pathol 16:14-9. 2013
    ..5, CTNNB1, WTX, TP53, FBXW7, and MYCN have also been linked to Wilms tumorigenesis...
  20. pmc Proteomic analysis of ubiquitin ligase KEAP1 reveals associated proteins that inhibit NRF2 ubiquitination
    Bridgid E Hast
    Department of Cell Biology and Physiology, Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC, USA
    Cancer Res 73:2199-210. 2013
    ..Previously, we and others discovered that via a competitive binding mechanism, the proteins WTX (AMER1), PALB2, and SQSTM1 bind KEAP1 to activate NRF2...
  21. pmc Wilms tumor gene on X chromosome (WTX) inhibits degradation of NRF2 protein through competitive binding to KEAP1 protein
    Nathan D Camp
    Howard Hughes Medical Institute, Department of Pharmacology, Institute for Stem Cell and Regenerative Medicine, University of Washington School of Medicine, Seattle, Washington 98195, USA
    J Biol Chem 287:6539-50. 2012
    b>WTX is a tumor suppressor protein that is lost or mutated in up to 30% of cases of Wilms tumor...
  22. doi Stat3 inhibits WTX expression through up-regulation of microRNA-370 in Wilms tumor
    Xu Cao
    Department of Surgery, Childrens Hospital Affiliated to Soochow University, Soochow University, Suzhou 215003, PR China
    FEBS Lett 587:639-44. 2013
    ..Several genetic mutations have been identified in WT patients, including inactivation of WTX, somatic stabilizing CTNNB1, and p53 mutations...
  23. pmc Clinically relevant subsets identified by gene expression patterns support a revised ontogenic model of Wilms tumor: a Children's Oncology Group Study
    Samantha Gadd
    Department of Pathology, Northwestern University s Feinberg School of Medicine and Robert H Lurie Cancer Center, Chicago, IL, USA
    Neoplasia 14:742-56. 2012
    ..We evaluated 224 WT for global gene expression patterns; WT1, CTNNB1, and WTX mutation; and 11p15 copy number and methylation patterns...
  24. pmc FAM123A binds to microtubules and inhibits the guanine nucleotide exchange factor ARHGEF2 to decrease actomyosin contractility
    Priscila F Siesser
    Department of Cell and Developmental Biology, Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC 27599, USA
    Sci Signal 5:ra64. 2012
    The FAM123 gene family comprises three members: FAM123A, the tumor suppressor WTX (also known as FAM123B), and FAM123C...
  25. pmc WTX R353X mutation in a family with osteopathia striata and cranial sclerosis (OS-CS): case report and literature review of the disease clinical, genetic and radiological features
    Anna Maria Zicari
    Department of Pediatrics, Policlinico Umberto I, Sapienza University, Rome, Italy
    Ital J Pediatr 38:27. 2012
    ..b>WTX gene (Xq11) has been recently identified as the disease causing gene...
  26. pmc Stratification of Wilms tumor by genetic and epigenetic analysis
    Richard H Scott
    Division of Genetics and Epidemiology, Institute of Cancer Research and Royal Marsden Hospital, Sutton, UK
    Oncotarget 3:327-35. 2012
    Somatic defects at five loci, WT1, CTNNB1, WTX, TP53 and the imprinted 11p15 region, are implicated in Wilms tumor, the commonest childhood kidney cancer. In this study we analysed all five loci in 120 Wilms tumors...
  27. doi Genetics of pediatric renal tumors
    Brigitte Royer-Pokora
    Institute for Human Genetics and Anthropology, Medical Faculty, Heinrich Heine University, Moorenstrasse 5, 40225 Dusseldorf, Germany
    Pediatr Nephrol 28:13-23. 2013
    ..Other genes frequently altered somatically in subsets of WT are CTNNB1 and WTX; both genes influence the Wnt signalling pathway...
  28. doi Severe osteopathia striata with cranial sclerosis in a female case with whole WTX gene deletion
    Sean B Herman
    Division of Plastic and Reconstructive Surgery, Department of Surgery, Montefiore Medical Center, Bronx, NY 10467, USA
    Am J Med Genet A 161:594-9. 2013
    Osteopathia striata with cranial sclerosis (OSCS) is caused by truncating mutations or deletions in the X linked gene, WTX, and is characterized by sclerotic striations of the metaphyses and diaphyses of long bones, pelvis, and scapula, ..
  29. pmc WTX: an unexpected regulator for p53
    Chao Dai
    Institute for Cancer Genetics, College of Physicians and Surgeons, Columbia University, 1130 Saint Nicholas Avenue, New York, NY 10032, USA
    Mol Cell 45:581-2. 2012
    The WTX gene is frequently lost or mutated in Wilms tumor. In this issue of Molecular Cell, Kim et al. (2012) identify WTX modulation of p53 tumor-suppressor activity through regulation of p53 acetylation...
  30. pmc The WTX tumor suppressor enhances p53 acetylation by CBP/p300
    Woo Jae Kim
    Massachusetts General Hospital Cancer Center, Harvard Medical School, Charlestown, MA 02129, USA
    Mol Cell 45:587-97. 2012
    b>WTX encodes a tumor suppressor, frequently inactivated in Wilms tumor, with both plasma membrane and nuclear localization. WTX has been implicated in β-catenin turnover, but its effect on nuclear proteins is unknown...
  31. doi Different incidences of epigenetic but not genetic abnormalities between Wilms tumors in Japanese and Caucasian children
    Masayuki Haruta
    Department of Cancer Diagnosis, Research Institute for Clinical Oncology, Saitama Cancer Center, Saitama, Japan
    Cancer Sci 103:1129-35. 2012
    ..Abnormalities of WT1, CTNNB1, WTX, and IGF2 were reported to be involved in Wilms tumorigenesis in Caucasians, although none of the studies ..
  32. ncbi Weak neurotoxin from Naja kaouthia cobra venom affects haemodynamic regulation by acting on acetylcholine receptors
    Alexey Ya Ogay
    Branch of Shemyakin and Ovchinnikov Institute of Bioorganic Chemistry RAS, Pushchino, Moscow Region, Russia
    Toxicon 45:93-9. 2005
    ..We have studied the influence of weak neurotoxin (WTX) from the venom of cobra Naja kaouthia on arterial blood pressure (BP) and heart rate (HR) in rats and mice...
  33. ncbi [Structure and conformational heterogeneity of the weak toxin from the cobra Naja kaouthia venom]
    A V Eletskiĭ
    Shemyakin Ovchinnikov Institute of Bioorganic Chemistry, Russian Academy of Sciences, ul Miklukho Maklaya 16 10, GSP Moscow, 117997 Russia
    Bioorg Khim 27:89-101. 2001
    Resonances in the two-dimensional 1H NMR spectra of a weak toxin (WTX) from the venom of cobra Naja kaouthia for all 65 amino acid residues were assigned...
  34. ncbi Direct cloning of a target gene from a pool of homologous sequences: complete cDNA sequence of a weak neurotoxin from cobra Naja kaouthia
    T L Oustitch
    Eppendorf 5 Prime, Inc, 6531 Gunbarrel Av, Boulder, CO 80301, USA
    IUBMB Life 55:43-7. 2003
    Selective cloning of the cDNA coding for a weak neurotoxin (WTX) from cobra N. kaouthia including the 5'- and 3'-non-translated regions (NTR) is described...
  35. ncbi Residues in the epsilon subunit of the nicotinic acetylcholine receptor interact to confer selectivity of waglerin-1 for the alpha-epsilon subunit interface site
    Brian E Molles
    Department of Pharmacology, Biomedical Sciences Graduate Program, University of California at San Diego, La Jolla, CA92093 0636, USA
    Biochemistry 41:7895-906. 2002
    Waglerin-1 (Wtx-1) is a 22-amino acid peptide that competitively antagonizes muscle nicotinic acetylcholine receptors (nAChRs)...
  36. ncbi First tryptophan-containing weak neurotoxin from cobra venom
    Y N Utkin
    Shemyakin Ovchinnikov Institute of Bioorganic Chemistry, Russian Academy of Sciences, Miklukho Maklaya 16 10, GSP 7 V 437, 11781, Moscow, Russia
    Toxicon 39:921-7. 2001
    ..relationships among weak neurotoxins (called so because of their low toxicity), we have isolated a toxin (WTX) from the venom of cobra Naja kaouthia using a combination of gel-filtration and ion-exchange chromatography...
  37. doi Tumor suppressor WTX gene mutation is rare in acute leukemias
    Nak Gyun Chung
    Leuk Lymphoma 49:1616-7. 2008
  38. pmc Amer2 protein is a novel negative regulator of Wnt/β-catenin signaling involved in neuroectodermal patterning
    Astrid S Pfister
    Nikolaus Fiebiger Center for Molecular Medicine, University Erlangen Nuremberg, 91054 Erlangen, Germany
    J Biol Chem 287:1734-41. 2012
    ..APC membrane recruitment 2; FAM123A) is a direct interaction partner of APC, related to the tumor suppressor Amer1/WTX, but its function in Wnt signaling is not known...
  39. ncbi The male phenotype in osteopathia striata congenita with cranial sclerosis
    Sarah K Holman
    Department of Paediatrics, Dunedin School of Medicine, Otago University, New Zealand
    Am J Med Genet A 155:2397-408. 2011
    Osteopathia striata with cranial sclerosis (OSCS) is an X-linked disease caused by truncating mutations in WTX. Females exhibit sclerotic striations on the long bones, cranial sclerosis, and craniofacial dysmorphism...
  40. pmc β-Catenin and K-RAS synergize to form primitive renal epithelial tumors with features of epithelial Wilms' tumors
    Peter E Clark
    Department of Urologic Surgery, Vanderbilt University Medical Center, Nashville, Tennessee 37232 2765, USA
    Am J Pathol 179:3045-55. 2011
    ..Wilms' tumor (WT) is the most common childhood renal cancer. Although mutations in known tumor-associated genes (WT1, WTX, and CATNB) occur only in a third of tumors, many tumors show evidence of activated β-catenin-dependent Wnt ..
  41. doi Canonical WNT signalling determines lineage specificity in Wilms tumour
    R Fukuzawa
    Cancer Genetics Laboratory, Department of Biochemistry, University of Otago, Dunedin, New Zealand
    Oncogene 28:1063-75. 2009
    ..In addition, we screened for mutations in WTX, which has been proposed to be a negative regulator of the canonical WNT-signalling pathway...
  42. ncbi [Osteopathia striata with cranial sclerosis]
    Mafalda Barbosa
    Centro de Genética Médica Dr Jacinto Magalhães, Departamento de Genetica, Instituto Nacional de Saude Dr Ricardo Jorge, Porto
    Acta Med Port 23:1147-50. 2010
    ..The radiological findings led to the diagnosis of Osteopathia Striata with Cranial Sclerosis. A mutation in WTX gene confirmed the clinical and radiological diagnosis of Osteopathia Striata with Cranial Sclerosis in this ..
  43. doi The WTX tumor suppressor regulates mesenchymal progenitor cell fate specification
    Annie Moisan
    Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA 02114, USA
    Dev Cell 20:583-96. 2011
    b>WTX is an X-linked tumor suppressor targeted by somatic mutations in Wilms tumor, a pediatric kidney cancer, and by germline inactivation in osteopathia striata with cranial sclerosis, a bone overgrowth syndrome...
  44. doi Pediatric genitourinary tumors
    Thomas W McLean
    Department of Pediatrics, Wake Forest University School of Medicine, Winston Salem, North Carolina 27157, USA
    Curr Opin Oncol 20:315-20. 2008
    ..We will review the recent (2006/2007) literature on pediatric genitourinary tumors...
  45. ncbi [The tumor suppressor gene WTX, mutated in Wilms tumours, is a member of the beta-catenin destruction complex]
    Stephane Angers
    Chaire de Recherche du Canada en Architecture Fonctionnelle des Complexes de Signalisation, Leslie Dan Faculty of Pharmacy, University of Toronto, Toronto, Ontario, M5S 3M2 Canada
    Med Sci (Paris) 23:1025-7. 2007
  46. ncbi Wilms' tumor with an apparently balanced translocation t(X;18) resulting in deletion of the WTX gene
    Moonjoo Han
    Molecular Diagnostics Laboratory, Department of Pathology, Massachusetts General Hospital, and Harvard Medical School, Boston, MA 02114, USA
    Genes Chromosomes Cancer 46:909-13. 2007
    The recent description of a new X chromosome tumor suppressor gene, WTX, that is commonly inactivated in Wilms' tumor prompted us to examine the possible involvement of WTX in a case of Wilms' tumor containing an apparently balanced ..
  47. pmc X-linked tumor suppressors: perplexing inheritance, a unique therapeutic opportunity
    Yang Liu
    Divisions of Immunotherapy, Department of Surgery, University of Michigan School of Medicine, Ann Arbor, MI 48105, USA
    Trends Genet 26:260-5. 2010
    ..Recent studies have identified FOXP3 and WTX as two X-linked tumor suppressor genes that are somatically inactivated by single genetic hits...
  48. ncbi Wilms tumor genetics: a new, UnX-pected twist to the story
    Vicki Huff
    Department of Cancer Genetics, Unit 1010, University of Texas M D Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA
    Cancer Cell 11:105-7. 2007
    ..The discovery of an X chromosome gene, WTX, that is mutated somatically in approximately 30% of Wilms tumors is notable both for helping to explain the ..
  49. pmc Genetic differentiation among geographic populations of Gonatocerus ashmeadi, the predominant egg parasitoid of the glassy-winged sharpshooter, Homalodisca coagulata
    Jesse H De León
    United States Department of Agriculture, Agricultural Research Service, Subtropical Agricultural Research Center, Beneficial Insects Research Unit, 2413 E Highway 83, Weslaco, Texas 78596, USA
    J Insect Sci 5:2. 2005
    ..ashmeadi were analyzed. Four populations (California; San Antonio, Texas; Weslaco, Texas [WTX-2]; and Florida) were field collected and two (Louisiana and Weslaco, Texas [WTX-1]) were reared...
  50. ncbi Computer modeling of binding of diverse weak toxins to nicotinic acetylcholine receptors
    D Yu Mordvitsev
    Shemyakin Ovchinnikov Institute of Bioorganic Chemistry RAS, Miklukho Maklaya str, 16 10, GSP 7, 117997 Moscow, Russia
    Comput Biol Chem 31:72-81. 2007
    ..simulations are used for comparative modeling of the complexes between four weak toxins of known spatial structure (WTX, candoxin, bucandin, gamma-bungarotoxin) and nAChRs...
  51. ncbi Identification of residues at the alpha and epsilon subunit interfaces mediating species selectivity of Waglerin-1 for nicotinic acetylcholine receptors
    Brian E Molles
    Department of Pharmacology, University of California, San Diego, La Jolla, California 92093 0636, USA
    J Biol Chem 277:5433-40. 2002
    Waglerin-1 (Wtx-1) is a 22-amino acid peptide that is a competitive antagonist of the muscle nicotinic receptor (nAChR)...
  52. ncbi Nicotinic receptors in Lymnaea stagnalis neurons are blocked by alpha-neurotoxins from cobra venoms
    C A Vulfius
    lnstitute of Cell Biophysics, Russian Academy of Sciences, Pushchino, Moscow Region, 142290, Russia
    Neurosci Lett 309:189-92. 2001
    ..It was found that a short chain neurotoxin II (NT II), a long chain cobratoxin (CTX) and weak neurotoxin (WTX) diminished the ACh-induced currents, the block being concentration-dependent and competitive...
  53. doi Clinical and molecular description of a Wilms tumor in a patient with tuberous sclerosis complex
    Filippo Spreafico
    Pediatric Oncology Unit, Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
    Am J Med Genet A 155:1419-24. 2011
    ..Deletion of the WTX gene was also present, but it involved the functionally inactive X chromosome...
  54. doi Pathology, genetics and cytogenetics of Wilms' tumour
    Reena Md Zin
    School of Pathology and Laboratory Medicine, University of Western Australia, QEII Medical Centre, Nedlands, Western Australia, Australia
    Pathology 43:302-12. 2011
    ..have improved our ability to characterise changes in genes involved in WT which include WT1, CTNNB1, IGF2 and WTX. These genetic events have not only provided insight into the pathobiology of this malignancy, but the recognition ..
  55. pmc Mutations in the WTX-gene are found in some high-grade microsatellite instable (MSI-H) colorectal cancers
    Silvio K Scheel
    Pathologisches Institut der Ludwig Maximilians Universität München, Thalkirchner Strasse 36, 80337 Munich, Germany
    BMC Cancer 10:413. 2010
    ..Recently in Wilms tumors, WTX (Wilms tumor gene on the X-chromosome) was discovered as another gene involved in the destruction of beta-CATENIN...
  56. doi WNT/beta-catenin pathway activation in Wilms tumors: a unifying mechanism with multiple entries?
    Marie Corbin
    INSERM, U574, Hopital Necker Enfants Malades, Paris, France
    Genes Chromosomes Cancer 48:816-27. 2009
    ..We characterized mutations of the WNT pathway regulator gene WTX in 16% of this tumor class...
  57. doi Expression patterns of the Wtx/Amer gene family during mouse embryonic development
    Glenda Comai
    Inserm U636, Centre de Biochimie, and University of Nice Sophia Antipolis, Nice, France
    Dev Dyn 239:1867-78. 2010
    ..WTX/AMER1 (Fam123b) shares several domains of homology with two other recently identified proteins: AMER2 (Fam123a) and AMER3 (..
  58. doi Weak toxin WTX from Naja kaouthia cobra venom interacts with both nicotinic and muscarinic acetylcholine receptors
    Dmitry Yu Mordvintsev
    Shemyakin Ovchinnikov Institute of Bioorganic Chemistry RAS, Moscow, Russia
    FEBS J 276:5065-75. 2009
    Iodinated [125I] weak toxin from Naja kaouthia (WTX) cobra venom was injected into mice, and organ-specific binding was monitored. Relatively high levels of [125I]WTX were detected in the adrenal glands...
  59. doi Subtype-specific FBXW7 mutation and MYCN copy number gain in Wilms' tumor
    Richard D Williams
    Section of Paediatric Oncology, Institute of Cancer Research, Sutton, Surrey, United Kingdom
    Clin Cancer Res 16:2036-45. 2010
    ..renal malignancy, is associated with mutations in several well-characterized genes, most notably WT1, CTNNB1, WTX, and TP53. However, the majority of cases do not harbor mutations in these genes...
  60. doi Is predisposition for nephroblastoma linked to polymorphisms of the WTX gene?
    Barbara Guertl
    Institute of Pathology, Medical University of Graz, Auenbruggerplatz 25, A 8036, Graz, Austria
    Pathol Oncol Res 16:189-91. 2010
    Inactivation of Wilms tumor X (WTX) gene has been linked to the pathogenesis of a varying percentage of nephroblastomas...
  61. doi Pediatric genitourinary tumors
    Thomas W McLean
    Department of Pediatrics, Wake Forest University School of Medicine, Winston Salem, North Carolina 27157, USA
    Curr Opin Oncol 22:268-73. 2010
    ..To review the 2008-2009 literature on pediatric genitourinary tumors and highlight the most significant publications...
  62. pmc Mosaicism in osteopathia striata with cranial sclerosis
    Dennis J Joseph
    Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana 46202 5121, USA
    J Clin Endocrinol Metab 95:1506-7. 2010
    Osteopathia striata with cranial sclerosis is an X-linked dominant condition caused by mutations in the WTX gene, resulting in linear striations in long bones in combination with cranial sclerosis...
  63. pmc Randomised, non-comparative phase II study of weekly docetaxel with cisplatin and 5-fluorouracil or with capecitabine in oesophagogastric cancer: the AGITG ATTAX trial
    N C Tebbutt
    Department of Medical Oncology, Austin Health, PO Box 5555, Studley Road, Heidelberg, Melbourne, Victoria 3084, Australia
    Br J Cancer 102:475-81. 2010
    ..Weekly docetaxel is associated with less haematological toxicity. This randomised phase II study tested weekly docetaxel-based combination chemotherapy regimens, with the aim of maintaining their activity while reducing toxicity...
  64. pmc The WTX/AMER1 gene family: evolution, signature and function
    Agnes Boutet
    INSERM, U636, F 06108 Nice, France
    BMC Evol Biol 10:280. 2010
    b>WTX is a novel gene mutated in a proportion of Wilms' tumors and in patients suffering from sclerosing bone dysplasia...
  65. ncbi [New weak toxins from the cobra venom.]
    V G Starkov
    Shemyakin Ovchinnikov Institute of Bioorganic Chemistry, Russian Academy of Sciences, ul Miklukho Maklaya 16 10, Moscow, 117997, Russia
    Bioorg Khim 35:15-24. 2009
    ..mass spectrometry has shown that one of these proteins is a novel weak toxin and the other is the known weak toxin WTX with an oxidized methionine residue in position 9...
  66. pmc Structural and functional characterization of the Wnt inhibitor APC membrane recruitment 1 (Amer1)
    Kristina Tanneberger
    Nikolaus Fiebiger Center, Biology Department, University Erlangen Nuremberg, Erlangen, Germany
    J Biol Chem 286:19204-14. 2011
    Amer1/WTX binds to the tumor suppressor adenomatous polyposis coli and acts as an inhibitor of Wnt signaling by inducing β-catenin degradation...
  67. doi Resistance or sensitivity of Wilms' tumor to anti-FZD7 antibody highlights the Wnt pathway as a possible therapeutic target
    N Pode-Shakked
    Pediatric Stem Cell Research Institute, Safra Children s Hospital and Sheba Center for Regenerative Medicine, Sheba Medical Center, Tel Hashomer, Israel
    Oncogene 30:1664-80. 2011
    ..Wnt inhibitors) expression pattern, restored epigenetically with de-methylating agents, and lack of β-catenin or WTX mutations...
  68. ncbi [Establishment of BGC-823/WTX-EGFP gastric cancer cell line stably expressing Wilms tumor gene on X chromosome]
    Ke xu CHENG
    Department of Pathology, Nanfang Hospital, Southern Medical University, College of Basic Medicine, Guangzhou 510515, China
    Nan Fang Yi Ke Da Xue Xue Bao 31:392-6. 2011
    To establish the BGC-823/WTX-EGFP gastric cancer cell line with stable expression of Wilms tumor gene on the X chromosome (MTX) for functional analysis of WTX gene.
  69. doi LMP1 antagonizes WNT/β-catenin signalling through inhibition of WTX and promotes nasopharyngeal dysplasia but not tumourigenesis in LMP1(B95-8) transgenic mice
    Zhang Qingling
    Department of Pathology, Nanfang Hospital, Southern Medical University, Guangzhou, PR China
    J Pathol 223:574-83. 2011
    ..This pathway is antagonized by WTX (Wilms' tumour gene on the X chromosome), which can promote the ubiquitination and degradation of β-catenin...
  70. pmc Inheritance of susceptibility to induction of nephroblastomas in the Noble rat
    Bhalchandra A Diwan
    Basic Research Program, Science Applications International Corporation Frederick, Inc, National Cancer Institute, Frederick, MD 21702, USA
    Differentiation 77:424-32. 2009
    ..Since established Wilms tumor-associated suppressor genes, Wt1 and Wtx, were not mutated in normal or neoplastic tissues, genomic profiling was performed on isolated Nb and F344 ..
  71. doi Wilms' tumours: about tumour suppressor genes, an oncogene and a chameleon gene
    Vicki Huff
    Department of Genetics, University of Texas MD Anderson Cancer Center, Houston, Texas 77030, USA
    Nat Rev Cancer 11:111-21. 2011
    ..tumour include TP53, a classic tumour suppressor gene (TSG); CTNNB1 (encoding β-catenin), a classic oncogene; WTX, which accumulating data indicate is a TSG; and WT1, which is inactivated in some Wilms' tumours, similar to a TSG...
  72. pmc WT1 mutation and 11P15 loss of heterozygosity predict relapse in very low-risk wilms tumors treated with surgery alone: a children's oncology group study
    Elizabeth J Perlman
    Children s Memorial Hospital, 2300 Children s Plaza, Box 17, Chicago IL 60614, USA
    J Clin Oncol 29:698-703. 2011
    ..The current study validates these findings within prospectively identified children with VLRWT who did not receive adjuvant chemotherapy...
  73. doi Inactivation of the APC gene is constant in adrenocortical tumors from patients with familial adenomatous polyposis but not frequent in sporadic adrenocortical cancers
    Sebastien Gaujoux
    Institut Cochin, Université Paris Descartes Faculté de Médecine, CNRS UMR 8104, Paris, France
    Clin Cancer Res 16:5133-41. 2010
    ..germline APC mutations, as well as in patients with Beckwith-Wiedemann syndrome with Wilms' tumors reported to have WTX somatic mutations...
  74. doi Wilms tumor arising in a child with X-linked nephrogenic diabetes insipidus
    Reyhan El-Kares
    Department of Pediatrics, Montreal Children s Hospital Research Institute, 4060 Ste Catherine West, Montreal, Quebec, Canada
    Pediatr Nephrol 24:1313-9. 2009
    ..Wilms tumor is also genetically heterogeneous and is associated with mutations of WT1 (15-20%), WTX (20-30%) and other loci...
  75. doi WTX inactivation is a frequent, but late event in Wilms tumors without apparent clinical impact
    Jenny Wegert
    Developmental Biochemistry, Biocenter, University of Wuerzburg, Germany
    Genes Chromosomes Cancer 48:1102-11. 2009
    ..Mutations in WT1 and CTNNB1 are well established as causal alterations in about 10-15% of cases. Recently, WTX (WT gene on the X-chromosome), a gene implicated in WNT signaling, has been identified as a third WT gene...

Research Grants60

  1. Tumor burden and T cell immunity
    Yang Liu; Fiscal Year: 2006
    ..This will not only reveal the basic principles of anti-tumor immune response, but will also allow us to rationally design T cell immunotherapy. ..
  2. Functional Characterization of WTX in Renal Development
    Miguel Rivera; Fiscal Year: 2007
    ..The candidate for this KO8 award, Dr. Miguel Rivera, has identified and cloned a novel tumor suppressor gene, WTX, which is inactivated in 30% of sporadic Wilms tumor cases...
  3. PEDIATRIC ONCOLOGY GROUP STUDIES
    Thomas McLean; Fiscal Year: 2002
    ....
  4. TRAIL Mediated Apoptosis in Renal Cell Carcinoma
    Peter Clark; Fiscal Year: 2007
    ..abstract_text> ..
  5. CD24 Polymorphism and multiple sclerosis
    Yang Liu; Fiscal Year: 2007
    ..Our results will have important implications for the diagnosis and treatment of MS. ..
  6. Checkpoints for homeostatsis and autoimmune diseases
    Yang Liu; Fiscal Year: 2007
    ..Targeting this checkpoint may provide a novel therapeutic approach for autoimmune diseases that involve abnormal homeostatic proliferation. ..
  7. Checkpoints of Host Response to Cellular Injuries
    Yang Liu; Fiscal Year: 2010
    ..Since CD24 defects block the development of autoimmune diseases, our study provides a molecular basis linking inflammation to immunity to cancer or infection without provoking autoimmune diseases. ..
  8. CD24 in Cancer Resistance and Immunotherapy
    Yang Liu; Fiscal Year: 2011
    ..The results may also lead to practical approaches to enhancing efficacy of cancer immunotherapy. ..
  9. FoxP3 as the X-Linked Breast Cancer Suppressor Gene
    Yang Liu; Fiscal Year: 2009
    ....
  10. Checkpoints of Host Response to Cellular Injuries
    Yang Liu; Fiscal Year: 2010
    ..Since CD24 defects block the development of autoimmune diseases, our study provides a molecular basis linking inflammation to immunity to cancer or infection without provoking autoimmune diseases. ..
  11. FoxP3 as the X-Linked Breast Cancer Suppressor Gene
    Yang Liu; Fiscal Year: 2010
    ....
  12. CD24 in Cancer Resistance and Immunotherapy
    Yang Liu; Fiscal Year: 2010
    ..The results may also lead to practical approaches to enhancing efficacy of cancer immunotherapy. ..
  13. Selective Modulation of Cancer Immunity and Autoimmunity
    Yang Liu; Fiscal Year: 2010
    ..Moreover, our work also addresses fundamental issues on the specificity and function of regulatory T cells. ..
  14. Costimulation and Significance of Negative Selection
    Yang Liu; Fiscal Year: 2007
    ..abstract_text> ..
  15. COSTIMULATION FOR MEMORY AND EFFECTOR T CELLS
    Yang Liu; Fiscal Year: 2001
    ..Second, they will carry out lineage-ablation experiments to test whether effector T-cells are precursors of memory T-cells. ..
  16. RESPONSE AND TOLERANCE TO AN UNMUTATED TUMOR ANTIGEN PIA
    Yang Liu; Fiscal Year: 2001
    ....
  17. RECEPTORS FOR B7-MEDIATED T CELL EFFECTOR FUNCTIONS
    Yang Liu; Fiscal Year: 1999
    ..The experiments will employ site directed mutagenesis of B7-1 and the use of CD28 KO mice. ..
  18. Costimulation and Significance of Negative Selection
    Yang Liu; Fiscal Year: 2003
    ..abstract_text> ..
  19. B7 RECEPTORS, TUMOR IMMUNOTHERAPY AND IMMUNE REGULATION
    Yang Liu; Fiscal Year: 2003
    ..More importantly, the proposed studies will provide animal models that can be used to screen monoclonal antibodies that are of therapeutic value for human cancer. ..
  20. Selective Modulation of Cancer Immunity and Autoimmunity
    Yang Liu; Fiscal Year: 2006
    ..Moreover, our work also addresses fundamental issues on the specificity and function of regulatory T cells. ..
  21. CATEGORIZATION OF WILMS TUMOR BY GENETIC EXPRESSION
    Elizabeth Perlman; Fiscal Year: 2006
    ..Genes predictive of molecular categories will be verified using in situ hybridization or immunohistochemistry. A model categorization will be proposed and tested on 200 additional Wilms tumors. ..
  22. Costimulation and Significance of Negative Selection
    Yang Liu; Fiscal Year: 2006
    ..abstract_text> ..