Literature DB >> 30510842

Close yet so far away: a look into the management strategies of genetic imprinting disorders.

Mark A Pianka1, Alec T McIntosh1, Sahaj D Patel1, Pegah R Bakhshi1, Mira Jung2.   

Abstract

Genetic imprinting is the process of epigenetic labelling or silencing of particular genes, based on the maternal or paternal origin of the gene, in a heritable pattern. The incidence of imprinting disorders has become a growing concern due to the potential association between these congenital syndromes and assisted reproductive technologies (ARTs). This review presents a general summary of the imprinting process as well as the current knowledge surrounding the genetic and epigenetic underpinnings of the most prevalent imprinting disorders: Beckwith-Wiedemann syndrome (BWS), Silver-Russell syndrome (SRS), Prader-Willi syndrome (PWS), and Angelman syndrome (AS). As research continues to elucidate the molecular pathways that characterize genetic imprinting, efforts have been made to establish guidelines that incorporate phenotypic manifestations as well as genetic testing to ensure safe and effective management of symptoms. While these efforts are likely to benefit future clinical management, their efficacy cannot yet be generalized to all patients diagnosed with these syndromes, as many of the genetic abnormalities and the associated phenotypic manifestations have yet to be characterized. Furthermore, future advances in the knowledge of epigenetic processes and genetic loci involved in the development of these syndromes may allow for the development of curative therapies.

Entities:  

Keywords:  Epigenetics; angelman syndrome; beckwith-wiedemann syndrome; imprinting; prader-willi syndrome; silver-russell syndrome

Year:  2018        PMID: 30510842      PMCID: PMC6261869     

Source DB:  PubMed          Journal:  Am J Stem Cells        ISSN: 2160-4150


  34 in total

1.  Loss of imprinting of a paternally expressed transcript, with antisense orientation to KVLQT1, occurs frequently in Beckwith-Wiedemann syndrome and is independent of insulin-like growth factor II imprinting.

Authors:  M P Lee; M R DeBaun; K Mitsuya; H L Galonek; S Brandenburg; M Oshimura; A P Feinberg
Journal:  Proc Natl Acad Sci U S A       Date:  1999-04-27       Impact factor: 11.205

Review 2.  The impact of assisted reproductive technologies on genomic imprinting and imprinting disorders.

Authors:  Asli Uyar; Emre Seli
Journal:  Curr Opin Obstet Gynecol       Date:  2014-06       Impact factor: 1.927

3.  Wiedemann-Beckwith syndrome: presentation of clinical and cytogenetic data on 22 new cases and review of the literature.

Authors:  M J Pettenati; J L Haines; R R Higgins; R S Wappner; C G Palmer; D D Weaver
Journal:  Hum Genet       Date:  1986-10       Impact factor: 4.132

Review 4.  Silver-Russell syndrome: genetic basis and molecular genetic testing.

Authors:  Thomas Eggermann; Matthias Begemann; Gerhard Binder; Sabrina Spengler
Journal:  Orphanet J Rare Dis       Date:  2010-06-23       Impact factor: 4.123

5.  The Use of Oxytocin to Improve Feeding and Social Skills in Infants With Prader-Willi Syndrome.

Authors:  Maïthé Tauber; Kader Boulanouar; Gwenaelle Diene; Sophie Çabal-Berthoumieu; Virginie Ehlinger; Pascale Fichaux-Bourin; Catherine Molinas; Sandy Faye; Marion Valette; Jeanne Pourrinet; Catie Cessans; Sylvie Viaux-Sauvelon; Céline Bascoul; Antoine Guedeney; Patric Delhanty; Vincent Geenen; Henri Martens; Françoise Muscatelli; David Cohen; Angèle Consoli; Pierre Payoux; Catherine Arnaud; Jean-Pierre Salles
Journal:  Pediatrics       Date:  2017-02       Impact factor: 7.124

6.  Prevalence of Beckwith-Wiedemann syndrome in North West of Italy.

Authors:  Alessandro Mussa; Silvia Russo; Agostina De Crescenzo; Nicoletta Chiesa; Cristina Molinatto; Angelo Selicorni; Lorenzo Richiardi; Lidia Larizza; Margherita Cirillo Silengo; Andrea Riccio; Giovanni Battista Ferrero
Journal:  Am J Med Genet A       Date:  2013-08-05       Impact factor: 2.802

7.  Epigenetic mutations of the imprinted IGF2-H19 domain in Silver-Russell syndrome (SRS): results from a large cohort of patients with SRS and SRS-like phenotypes.

Authors:  D Bartholdi; M Krajewska-Walasek; K Ounap; H Gaspar; K H Chrzanowska; H Ilyana; H Kayserili; I W Lurie; A Schinzel; A Baumer
Journal:  J Med Genet       Date:  2008-12-09       Impact factor: 6.318

8.  A maternal deletion upstream of the imprint control region 2 in 11p15 causes loss of methylation and familial Beckwith-Wiedemann syndrome.

Authors:  Jasmin Beygo; Ivana Joksic; Tim M Strom; Hermann-Josef Lüdecke; Julia Kolarova; Reiner Siebert; Zeljko Mikovic; Bernhard Horsthemke; Karin Buiting
Journal:  Eur J Hum Genet       Date:  2016-02-03       Impact factor: 4.246

9.  Towards a therapy for Angelman syndrome by targeting a long non-coding RNA.

Authors:  Linyan Meng; Amanda J Ward; Seung Chun; C Frank Bennett; Arthur L Beaudet; Frank Rigo
Journal:  Nature       Date:  2014-12-01       Impact factor: 49.962

Review 10.  Imprinting disorders: a group of congenital disorders with overlapping patterns of molecular changes affecting imprinted loci.

Authors:  Thomas Eggermann; Guiomar Perez de Nanclares; Eamonn R Maher; I Karen Temple; Zeynep Tümer; David Monk; Deborah J G Mackay; Karen Grønskov; Andrea Riccio; Agnès Linglart; Irène Netchine
Journal:  Clin Epigenetics       Date:  2015-11-14       Impact factor: 6.551

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  2 in total

Review 1.  CRISPR/Cas9 Epigenome Editing Potential for Rare Imprinting Diseases: A Review.

Authors:  Linn Amanda Syding; Petr Nickl; Petr Kasparek; Radislav Sedlacek
Journal:  Cells       Date:  2020-04-16       Impact factor: 6.600

2.  Intrachromosomal Looping and Histone K27 Methylation Coordinately Regulates the lncRNA H19-Fetal Mitogen IGF2 Imprinting Cluster in the Decidual Microenvironment of Early Pregnancy.

Authors:  Xue Wen; Qi Zhang; Lei Zhou; Zhaozhi Li; Xue Wei; Wang Yang; Jiaomei Zhang; Hui Li; Zijun Xu; Xueling Cui; Songling Zhang; Yufeng Wang; Wei Li; Andrew R Hoffman; Zhonghui Liu; Ji-Fan Hu; Jiuwei Cui
Journal:  Cells       Date:  2022-10-05       Impact factor: 7.666

  2 in total

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