Increased tumour risk for BWS patients correlates with aberrant H19 and not KCNQ1OT1 methylation: occurrence of KCNQ1OT1 hypomethylation in familial cases …

J Bliek, SM Maas, JM Ruijter… - Human molecular …, 2001 - academic.oup.com
Beckwith–Wiedemann syndrome (BWS) is an overgrowth malformation syndrome that maps
to human chromosome 11p15. 5, a region that harbours a number of imprinted genes. We …

Analysis of the methylation status of the KCNQ1OT and H19 genes in leukocyte DNA for the diagnosis and prognosis of Beckwith–Wiedemann syndrome

V Gaston, Y Le Bouc, V Soupre, L Burglen… - European journal of …, 2001 - nature.com
Beckwith–Wiedemann syndrome (BWS) is an overgrowth disorder involving developmental
abnormalities, tissue and organ hyperplasia and an increased risk of embryonal tumours …

Tumor development in the Beckwith–Wiedemann syndrome is associated with a variety of constitutional molecular 11p15 alterations including imprinting defects of …

R Weksberg, J Nishikawa, O Caluseriu… - Human molecular …, 2001 - academic.oup.com
Dysregulation of imprinted genes on human chromosome 11p15 has been implicated in
Beckwith–Wiedemann syndrome (BWS), an overgrowth syndrome associated with …

A maternally methylated CpG island in KvLQT1 is associated with an antisense paternal transcript and loss of imprinting in Beckwith–Wiedemann syndrome

NJ Smilinich, CD Day, GV Fitzpatrick… - Proceedings of the …, 1999 - National Acad Sciences
Loss of imprinting at IGF2, generally through an H19-independent mechanism, is associated
with a large percentage of patients with the overgrowth and cancer predisposition condition …

Wilms tumour in Beckwith–Wiedemann Syndrome and loss of methylation at imprinting centre 2: revisiting tumour surveillance guidelines

J Brzezinski, C Shuman, S Choufani, P Ray… - European Journal of …, 2017 - nature.com
Abstract Beckwith–Wiedemann Syndrome (BWS) is an overgrowth syndrome caused by a
variety of molecular changes on chromosome 11p15. 5. Children with BWS have a …

Epigenotyping as a tool for the prediction of tumor risk and tumor type in patients with Beckwith-Wiedemann syndrome (BWS)

J Bliek, C Gicquel, S Maas, V Gaston, Y Le Bouc… - The Journal of …, 2004 - Elsevier
OBJECTIVES: Patients with Beckwith-Wiedemann syndrome (BWS) have a risk of 7.5% to
10% of developing childhood tumors, 60% of which are Wilms' tumors. Aberrant methylation …

Imprinting status of 11p15 genes in Beckwith–Wiedemann syndrome patients with CDKN1C mutations

M Li, J Squire, C Shuman, J Atkin, R Pauli, A Smith… - Genomics, 2001 - Elsevier
Beckwith–Wiedemann syndrome (BWS) is an imprinting disorder characterized by somatic
overgrowth, congenital malformations, and predisposition to childhood tumors. Aberrant …

Silencing of CDKN1C (p57KIP2) is associated with hypomethylation at KvDMR1 in Beckwith–Wiedemann syndrome

N Diaz-Meyer, CD Day, K Khatod, ER Maher… - Journal of medical …, 2003 - jmg.bmj.com
Context: Beckwith–Wiedemann syndrome (BWS) arises by several genetic and epigenetic
mechanisms affecting the balance of imprinted gene expression in chromosome 11p15. 5 …

Epigenotype-phenotype correlations in Beckwith-Wiedemann syndrome

JR Engel, A Smallwood, A Harper… - Journal of medical …, 2000 - jmg.bmj.com
Beckwith-Wiedemann syndrome (BWS) is a model imprinting disorder resulting from
mutations or epigenetic events involving imprinted genes at chromosome 11p15. 5. Thus …

Molecular subtypes and phenotypic expression of Beckwith–Wiedemann syndrome

WN Cooper, A Luharia, GA Evans, H Raza… - European journal of …, 2005 - nature.com
Abstract Beckwith–Wiedemann Syndrome (BWS) results from mutations or epigenetic
events involving imprinted genes at 11p15. 5. Most BWS cases are sporadic and uniparental …