Genomes and Genes
Gene Symbol: WTX
Description: APC membrane recruitment protein 1
Alias: FAM123B, OSCS, WTX, APC membrane recruitment protein 1, RP11-403E24.2, 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
- An X chromosome gene, WTX, is commonly inactivated in Wilms tumorMiguel 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)...
- WTX is rarely mutated in acute myeloid leukemiaCarolyn Owen
Haematologica 93:947-8. 2008
- Functional inactivation of the WTX gene is not a frequent event in Wilms' tumorsD Perotti
Department of Experimental Oncology and Laboratories, Genetic Susceptibility to Cancer Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
Oncogene 27:4625-32. 2008For many years the precise genetic etiology of the majority of Wilms' tumors has remained unexplained. Recently, the WTX gene, mapped to chromosome Xq11...
- Mutational analysis of WTX gene in Wnt/ beta-catenin pathway in gastric, colorectal, and hepatocellular carcinomasNam 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. 2009A 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...
- Wilms tumor genetics: mutations in WT1, WTX, and CTNNB1 account for only about one-third of tumorsE 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...
- Germline mutations in WTX cause a sclerosing skeletal dysplasia but do not predispose to tumorigenesisZandra 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, ..
- AMER1 regulates the distribution of the tumor suppressor APC between microtubules and the plasma membraneAnnette 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...
- The tumor suppressor WTX shuttles to the nucleus and modulates WT1 activityMiguel N Rivera
Massachusetts General Hospital Cancer Center, Harvard Medical School, Charlestown, MA 20129, USA
Proc Natl Acad Sci U S A 106:8338-43. 2009b>WTX encodes a tumor suppressor gene inactivated in Wilms tumor and recently implicated in WNT signaling through enhancement of cytoplasmic beta-catenin (CTNNB1) degradation...
- Wilms tumor suppressor WTX negatively regulates WNT/beta-catenin signalingMichael 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-..
- Osteopathia striata with cranial sclerosis owing to WTX gene defectBram 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...
- WTX mutations can occur both early and late in the pathogenesis of Wilms tumourRyuji 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)...
- The WTX/AMER1 gene family: evolution, signature and functionAgnes Boutet
INSERM, U636, F 06108 Nice, France
BMC Evol Biol 10:280. 2010b>WTX is a novel gene mutated in a proportion of Wilms' tumors and in patients suffering from sclerosing bone dysplasia...
- Osteopathia striata cranial sclerosis: non-random X-inactivation suggestive of X-linked dominant inheritanceGeraldine 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...
- Functional characterization of Wilms tumor-suppressor WTX and tumor-associated mutantsM 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. 2011The WTX, Wilms tumor-associated tumor-suppressor gene, is present on the X chromosome and a single WTX mutation may be sufficient to promote carcinogenesis...
- 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. 2011Amer1/WTX binds to the tumor suppressor adenomatous polyposis coli and acts as an inhibitor of Wnt signaling by inducing β-catenin degradation...
- Amer1/WTX couples Wnt-induced formation of PtdIns(4,5)P2 to LRP6 phosphorylationKristina 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 ..
- MAP3K1 functionally interacts with Axin1 in the canonical Wnt signalling pathwaySer Sue Ng
Hubrecht Institute, KNAW and University Medical Centre Utrecht, Uppsalalaan 8, NL 3584 CT Utrecht, The Netherlands
Biol Chem 391:171-80. 2010..These results suggest that MAP3K1, previously reported as an Axin1 inter-actor in c-Jun NH(2)-terminal kinase pathway, is also involved in the canonical Wnt signalling pathway and positively regulates expression of Wnt target genes...
- Central loop of non-conventional toxin WTX from Naja kaouthia is important for interaction with nicotinic acetylcholine receptorsEkaterina N Lyukmanova
Lomonosov Moscow State University, Leninskie Gori 1, Moscow 119234, Russian Federation Shemyakin Ovchinnikov Institute of Bioorganic Chemistry RAS, Miklukho Maklaya Street 16 10, Moscow 117997, Russian Federation Electronic address
Toxicon 119:274-9. 2016Three-finger' toxin WTX from Naja kaouthia interacts with nicotinic and muscarinic acetylcholine receptors (nAChRs and mAChRs)...
- Osteopathia striata with cranial sclerosis and developmental delay in a male with a mosaic deletion in chromosome region Xq11.2Sébastien Chénier
The Hospital for Sick Children, Department of Paediatric Laboratory Medicine, Toronto, Ontario, Canada
Am J Med Genet A 158:2946-52. 2012Osteopathia striata with cranial sclerosis (OSCS) is an X-linked disease caused by mutations involving WTX (FAM123B), a tumor suppressor protein with dual functions...
- WT1, WTX and CTNNB1 mutation analysis in 43 patients with sporadic Wilms' tumorLeila C A Cardoso
Department of Genetics, Federal University of Rio de Janeiro, Rio de Janeiro, RJ, 21944 970, Brazil
Oncol Rep 29:315-20. 2013..Somatic mutations in exon 3 of CTNNB1, which encodes β-catenin, were initially observed in 15% of WT. WTX encodes a protein that negatively regulates the Wnt/β-catenin signaling pathway and mediates the ..
- Characterization of 17.94, a novel anaplastic Wilms' tumor cell lineKeith W Brown
University of Bristol, School of Cellular and Molecular Medicine, United Kingdom
Cancer Genet 205:319-26. 2012..a TP53 mutation, consistent with the anaplastic histology of the original tumor, but lacked mutations in WT1, WTX, or CTNNB1, which are the other genes involved in WT pathogenesis. The 17...
- Inactivation of the tumor suppressor WTX in a subset of pediatric tumorsSara Akhavanfard
Department of Pathology, Massachusetts General Hospital, Boston, MA Cancer Center, Massachusetts General Hospital, Boston, MA Harvard Medical School, Boston, MA
Genes Chromosomes Cancer 53:67-77. 2014b>WTX is a tumor suppressor gene expressed during embryonic development and inactivated in 20-30% of cases of Wilms tumor, the most common pediatric kidney cancer...
- Genetic variation frequencies in Wilms' tumor: A meta-analysis and systematic reviewChangkai Deng
Department of Urology Surgery, Children s Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorder, Key Laboratory of Pediatrics in Chongqing CSTC2009CA5002, Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Chongqing, China
Cancer Sci 107:690-9. 2016..0 software. Pooled prevalence for gene mutations WT1, WTX, CTNNB1, TP53, MYCN, DROSHA, and DGCR8 was 0.141 (0.104, 0.178), 0.147 (0.110, 0.184), 0.140 (0.100, 0.190), 0...
- Germline mosaicism in osteopathia striata with cranial sclerosis--recurrence in siblingsJames J O'Byrne
Departments of aClinical Genetics bRadiology, Our Lady s Children s Hospital Crumlin, Dublin, Ireland cDepartment of Medical Genetics, University of Antwerp, Antwerp, Belgium
Clin Dysmorphol 25:45-9. 2016We report recurrence of osteopathia striata with cranial sclerosis (OSCS) in two full siblings conceived by unaffected parents...
- Structural Insight into Specificity of Interactions between Nonconventional Three-finger Weak Toxin from Naja kaouthia (WTX) and Muscarinic Acetylcholine ReceptorsEkaterina N Lyukmanova
From the Shemyakin Ovchinnikov Institute of Bioorganic Chemistry, Russian Academy of Sciences, 16 10 Miklukho Maklaya Street, 117997 Moscow, Russia, The Lomonosov Moscow State University, 119991 Moscow, Russia
J Biol Chem 290:23616-30. 2015Weak toxin from Naja kaouthia (WTX) belongs to the group of nonconventional "three-finger" snake neurotoxins...
- Exome Sequencing Reveals AMER1 as a Frequently Mutated Gene in Colorectal CancerRebeca Sanz-Pamplona
Unit of Biomarkers and Susceptibility, Catalan Institute of Oncology ICO, Bellvitge Biomedical Research Institute IDIBELL and CIBERESP, L Hospitalet de Llobregat, Barcelona, Spain
Clin Cancer Res 21:4709-18. 2015..The aim of this study was to characterize the mutational landscape of stage II tumors and to search for novel recurrent mutations likely implicated in colorectal cancer tumorigenesis...
- The WTX Tumor Suppressor Interacts with the Transcriptional Corepressor TRIM28Woo Jae Kim
Massachusetts General Hospital Cancer Center and Harvard Medical School, Charlestown, Massachusetts 02129 and
J Biol Chem 290:14381-90. 2015b>WTX encodes a tumor suppressor implicated in the pediatric kidney cancer Wilms tumor and in mesenchymal differentiation with potentially distinct functions in the cytoplasm, at the plasma membrane, and in the nucleus...
- Polyclonal antibodies against native weak toxin Naja kaouthia discriminate native weak toxins and some other three-fingered toxins against their denaturated formsE V Kryukova
Shemyakin and Ovchinnikov Institute of Bioorganic Chemistry RAS, V 437, Miklukho Maklaya Street, 16 10, 117997 GSP, Moscow, Russia
Toxicon 46:24-30. 2005Polyclonal antibodies obtained by immunization of rabbits with native form of weak toxin (WTX) from cobra Naja kaouthia venom efficiently interacted with WTX and a weak toxin from Naja oxiana venom, but not so with their denaturated ..
- The development of Wilms tumor: from WT1 and microRNA to animal modelsFang Tian
Department of Pathophysiology, School of Basic Medicine, Zhengzhou University, Zhengzhou, Henan, PR China
Biochim Biophys Acta 1846:180-7. 2014..This has been illustrated by the findings that mutations of Wnt/β-catenin pathway-related WT1, β-catenin, and WTX together account for about one-third of Wilms tumor cases...
- Wilms tumor: an updateTurki Al-hussain
Departments of Pathology and Laboratory Medicine Pediatric Hematology Oncology, King Faisal Specialist Hospital and Research Centre, Riyadh, Kingdom of Saudi Arabia
Adv Anat Pathol 21:166-73. 2014..Abnormalities in several other genes may also be seen in WT. These include WTX, (on chromosome X), CTNNB1 (chromosome 3), and TP53 (chromosome 17) among others...
- Cancer-derived mutations in KEAP1 impair NRF2 degradation but not ubiquitinationBridgid E Hast
Authors Affiliations Department of Cell Biology and Physiology Lineberger Comprehensive Cancer Center and Division of Medical Oncology, Department of Internal Medicine and Otolaryngology, Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill School of Medicine Department of Computer Science, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina and Department of Pharmacology, Howard Hughes Medical Institute, University of Washington, Seattle, Washington
Cancer Res 74:808-17. 2014..These data strengthen the genetic interactions between KEAP1 and NRF2 in cancer and provide new insight into KEAP1 mechanics...
- β-arrestin promotes Wnt-induced low density lipoprotein receptor-related protein 6 (Lrp6) phosphorylation via increased membrane recruitment of Amer1 proteinVitezslav Kriz
From the Faculty of Science, Institute of Experimental Biology, Masaryk University, 611 37 Brno, Czech Republic
J Biol Chem 289:1128-41. 2014..via a novel interaction with phosphatidylinositol 4,5-bisphosphate (PtdIns(4,5)P2)-binding protein Amer1/WTX/Fam123b. Amer1 has been shown very recently to bridge Wnt-induced and Dishevelled-associated PtdIns(4,5)P2 production to ..
- A novel WTX mutation in a female patient with osteopathia striata with cranial sclerosis and hepatoblastomaAtsushi Fujita
Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan
Am J Med Genet A 164:998-1002. 2014Osteopathia striata with cranial sclerosis (OSCS) is an X-linked dominant sclerosing bone dysplasia...
- Genetics of pediatric renal tumorsBrigitte 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...
- [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. 2001Resonances 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...
- Gain of FAM123B and ARHGEF9 in an Obese Man with Intellectual Disability, Congenital Heart Defects and Multiple Supernumerary Ring ChromosomesR 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 ..
- Wilms tumor gene on X chromosome (WTX) inhibits degradation of NRF2 protein through competitive binding to KEAP1 proteinNathan 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. 2012b>WTX is a tumor suppressor protein that is lost or mutated in up to 30% of cases of Wilms tumor...
- The WTX tumor suppressor enhances p53 acetylation by CBP/p300Woo Jae Kim
Massachusetts General Hospital Cancer Center, Harvard Medical School, Charlestown, MA 02129, USA
Mol Cell 45:587-97. 2012b>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...
- WTX: an unexpected regulator for p53Chao 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. 2012The 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...
- Promoter methylation of Wilms' tumor gene on the X- chromosome in gastric cancerXia Liu
Department of Pathology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China
Nan Fang Yi Ke Da Xue Xue Bao 33:318-21. 2013To 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.
- Different incidences of epigenetic but not genetic abnormalities between Wilms tumors in Japanese and Caucasian childrenMasayuki 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 ..
- Defects in the DNA mismatch repair system do not contribute to the development of childhood wilms tumorsHeidi 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...
- Stratification of Wilms tumor by genetic and epigenetic analysisRichard H Scott
Division of Genetics and Epidemiology, Institute of Cancer Research and Royal Marsden Hospital, Sutton, UK
Oncotarget 3:327-35. 2012Somatic 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...
- Osteopathia striata congenita with cranial sclerosis and intellectual disability due to contiguous gene deletions involving the WTX locusS 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)...
- Severe osteopathia striata with cranial sclerosis in a female case with whole WTX gene deletionSean 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. 2013Osteopathia 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, ..
- 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 featuresAnna 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...
- Proteomic analysis of ubiquitin ligase KEAP1 reveals associated proteins that inhibit NRF2 ubiquitinationBridgid 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...
- FAM123A binds to microtubules and inhibits the guanine nucleotide exchange factor ARHGEF2 to decrease actomyosin contractilityPriscila 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. 2012The FAM123 gene family comprises three members: FAM123A, the tumor suppressor WTX (also known as FAM123B), and FAM123C...
- Clinically relevant subsets identified by gene expression patterns support a revised ontogenic model of Wilms tumor: a Children's Oncology Group StudySamantha 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...
- Stat3 inhibits WTX expression through up-regulation of microRNA-370 in Wilms tumorXu 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...
- Residues in the epsilon subunit of the nicotinic acetylcholine receptor interact to confer selectivity of waglerin-1 for the alpha-epsilon subunit interface siteBrian E Molles
Department of Pharmacology, Biomedical Sciences Graduate Program, University of California at San Diego, La Jolla, CA92093 0636, USA
Biochemistry 41:7895-906. 2002Waglerin-1 (Wtx-1) is a 22-amino acid peptide that competitively antagonizes muscle nicotinic acetylcholine receptors (nAChRs)...
- Direct cloning of a target gene from a pool of homologous sequences: complete cDNA sequence of a weak neurotoxin from cobra Naja kaouthiaT L Oustitch
Eppendorf 5 Prime, Inc, 6531 Gunbarrel Av, Boulder, CO 80301, USA
IUBMB Life 55:43-7. 2003Selective 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...
- Weak neurotoxin from Naja kaouthia cobra venom affects haemodynamic regulation by acting on acetylcholine receptorsAlexey 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...
- Two novel WTX mutations underscore the unpredictability of male survival in osteopathia striata with cranial sclerosisB 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)...
- [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
- 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. 2010Inactivation of Wilms tumor X (WTX) gene has been linked to the pathogenesis of a varying percentage of nephroblastomas...
- Weak toxin WTX from Naja kaouthia cobra venom interacts with both nicotinic and muscarinic acetylcholine receptorsDmitry Yu Mordvintsev
Shemyakin Ovchinnikov Institute of Bioorganic Chemistry RAS, Moscow, Russia
FEBS J 276:5065-75. 2009Iodinated [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...
- 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...
- [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...
- Wilms tumor arising in a child with X-linked nephrogenic diabetes insipidusReyhan 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...
- Inheritance of susceptibility to induction of nephroblastomas in the Noble ratBhalchandra 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 ..
- Canonical WNT signalling determines lineage specificity in Wilms tumourR 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...
- Tumor suppressor WTX gene mutation is rare in acute leukemiasNak Gyun Chung
Leuk Lymphoma 49:1616-7. 2008
- Pediatric genitourinary tumorsThomas 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...
- WTX inactivation is a frequent, but late event in Wilms tumors without apparent clinical impactJenny 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...
- Wilms' tumor with an apparently balanced translocation t(X;18) resulting in deletion of the WTX geneMoonjoo Han
Molecular Diagnostics Laboratory, Department of Pathology, Massachusetts General Hospital, and Harvard Medical School, Boston, MA 02114, USA
Genes Chromosomes Cancer 46:909-13. 2007The 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 ..
- Computer modeling of binding of diverse weak toxins to nicotinic acetylcholine receptorsD 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...
- Wilms tumor genetics: a new, UnX-pected twist to the storyVicki 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 ..
- Genetic differentiation among geographic populations of Gonatocerus ashmeadi, the predominant egg parasitoid of the glassy-winged sharpshooter, Homalodisca coagulataJesse 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...
- Identification of residues at the alpha and epsilon subunit interfaces mediating species selectivity of Waglerin-1 for nicotinic acetylcholine receptorsBrian E Molles
Department of Pharmacology, University of California, San Diego, La Jolla, California 92093 0636, USA
J Biol Chem 277:5433-40. 2002Waglerin-1 (Wtx-1) is a 22-amino acid peptide that is a competitive antagonist of the muscle nicotinic receptor (nAChR)...
- Nicotinic receptors in Lymnaea stagnalis neurons are blocked by alpha-neurotoxins from cobra venomsC 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...
- First tryptophan-containing weak neurotoxin from cobra venomY 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...
- Amer2 protein is a novel negative regulator of Wnt/β-catenin signaling involved in neuroectodermal patterningAstrid 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...
- [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. 2011To 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.
- Resistance or sensitivity of Wilms' tumor to anti-FZD7 antibody highlights the Wnt pathway as a possible therapeutic targetN 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...
- Wilms' tumours: about tumour suppressor genes, an oncogene and a chameleon geneVicki 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...
- Pathology, genetics and cytogenetics of Wilms' tumourReena 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 ..
- Clinical and molecular description of a Wilms tumor in a patient with tuberous sclerosis complexFilippo 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...
- The WTX tumor suppressor regulates mesenchymal progenitor cell fate specificationAnnie Moisan
Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA 02114, USA
Dev Cell 20:583-96. 2011b>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...
- [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 ..
- β-Catenin and K-RAS synergize to form primitive renal epithelial tumors with features of epithelial Wilms' tumorsPeter 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 ..
- The male phenotype in osteopathia striata congenita with cranial sclerosisSarah K Holman
Department of Paediatrics, Dunedin School of Medicine, Otago University, New Zealand
Am J Med Genet A 155:2397-408. 2011Osteopathia 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...
- LMP1 antagonizes WNT/β-catenin signalling through inhibition of WTX and promotes nasopharyngeal dysplasia but not tumourigenesis in LMP1(B95-8) transgenic miceZhang 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...
- WT1 mutation and 11P15 loss of heterozygosity predict relapse in very low-risk wilms tumors treated with surgery alone: a children's oncology group studyElizabeth 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...
- Inactivation of the APC gene is constant in adrenocortical tumors from patients with familial adenomatous polyposis but not frequent in sporadic adrenocortical cancersSebastien 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...
- Mutations in the WTX-gene are found in some high-grade microsatellite instable (MSI-H) colorectal cancersSilvio 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...
- Expression patterns of the Wtx/Amer gene family during mouse embryonic developmentGlenda 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 (..
- X-linked tumor suppressors: perplexing inheritance, a unique therapeutic opportunityYang 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...
- Subtype-specific FBXW7 mutation and MYCN copy number gain in Wilms' tumorRichard 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...
- Pediatric genitourinary tumorsThomas 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...
- Mosaicism in osteopathia striata with cranial sclerosisDennis J Joseph
Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana 46202 5121, USA
J Clin Endocrinol Metab 95:1506-7. 2010Osteopathia 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...
- Randomised, non-comparative phase II study of weekly docetaxel with cisplatin and 5-fluorouracil or with capecitabine in oesophagogastric cancer: the AGITG ATTAX trialN 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...
- David McDermott; Fiscal Year: 2016..Five major projects are supported through this application including: 1) Clinical Correlations of WTX Inactivation in Wilms Tumor;2) Validation of RCC biomarkers for early disease detection and response to targeted ..
- Functional Characterization of WTX in Renal DevelopmentMIGUEL NICOLAS RIVERA; Fiscal Year: 2011..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...
- PEDIATRIC ONCOLOGY GROUP STUDIESThomas McLean; Fiscal Year: 2002....
- COSTIMULATION FOR MEMORY AND EFFECTOR T CELLSYang Liu; Fiscal Year: 2001..Second, they will carry out lineage-ablation experiments to test whether effector T-cells are precursors of memory T-cells. ..
- B7 RECEPTORS, TUMOR IMMUNOTHERAPY AND IMMUNE REGULATIONYang 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. ..
- CATEGORIZATION OF WILMS TUMOR BY GENETIC EXPRESSIONElizabeth 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. ..
- Costimulation and Significance of Negative SelectionYang Liu; Fiscal Year: 2007..abstract_text> ..
- CD24 Polymorphism and multiple sclerosisYang Liu; Fiscal Year: 2007..Our results will have important implications for the diagnosis and treatment of MS. [unreadable] [unreadable]..
- Selective Modulation of Cancer Immunity and AutoimmunityYang Liu; Fiscal Year: 2010..Moreover, our work also addresses fundamental issues on the specificity and function of regulatory T cells. ..
- FoxP3 as the X-Linked Breast Cancer Suppressor GeneYang Liu; Fiscal Year: 2010....