Literature DB >> 31896775

Unraveling the genetic cause of hereditary ophthalmic disorders in Arab societies from Israel and the Palestinian Authority.

Anja K Mayer1,2, Ghassan Balousha3, Rajech Sharkia4,5, Muhammad Mahajnah6,7, Suhail Ayesh8, Martin Schulze9,2, Rebecca Buchert9, Ditta Zobor10, Abdussalam Azem11, Ludger Schöls12,13, Peter Bauer9, Bernd Wissinger14.   

Abstract

Visual impairment due to inherited ophthalmic disorders is amongst the most common disabilities observed in populations practicing consanguineous marriages. Here we investigated the molecular genetic basis of an unselected broad range of ophthalmic disorders in 20 consanguineous families from Arab villages of Israel and the Palestinian Authority. Most patients had little or very poor prior clinical workup and were recruited in a field study. Homozygosity mapping followed by candidate gene sequencing applying conventional Sanger sequencing or targeted next generation sequencing was performed in six families. In the remaining 14 families, one affected subject per family was chosen for whole exome sequencing. We discovered likely disease-causing variants, all homozygous, in 19 of 20 independent families (95%) including a previously reported novel disease gene for congenital nystagmus associated with foveal hypoplasia. Moreover, we found a family in which disease-causing variants for two collagenopathies - Stickler and Knobloch syndrome - segregate within a large sibship. Nine of the 19 distinct variants observed in this study were novel. Our study demonstrated a very high molecular diagnostic yield for a highly diverse spectrum of rare ophthalmic disorders in Arab patients from Israel and the Palestinian Authority, even with very limited prior clinical investigation. We conclude that 'genetic testing first' may be an economic way to direct clinical care and to support proper genetic counseling and risk assessment in these families.

Entities:  

Mesh:

Year:  2020        PMID: 31896775      PMCID: PMC7253446          DOI: 10.1038/s41431-019-0566-3

Source DB:  PubMed          Journal:  Eur J Hum Genet        ISSN: 1018-4813            Impact factor:   4.246


  54 in total

1.  Predicting the effects of coding non-synonymous variants on protein function using the SIFT algorithm.

Authors:  Prateek Kumar; Steven Henikoff; Pauline C Ng
Journal:  Nat Protoc       Date:  2009-06-25       Impact factor: 13.491

Review 2.  Next generation sequencing and the future of genetic diagnosis.

Authors:  Katja Lohmann; Christine Klein
Journal:  Neurotherapeutics       Date:  2014-10       Impact factor: 7.620

3.  MutationTaster2: mutation prediction for the deep-sequencing age.

Authors:  Jana Marie Schwarz; David N Cooper; Markus Schuelke; Dominik Seelow
Journal:  Nat Methods       Date:  2014-04       Impact factor: 28.547

Review 4.  The molecular basis of human retinal and vitreoretinal diseases.

Authors:  Wolfgang Berger; Barbara Kloeckener-Gruissem; John Neidhardt
Journal:  Prog Retin Eye Res       Date:  2010-03-31       Impact factor: 21.198

5.  Enamel surface modification in vitro using hydrochloric acid pumice: an SEM investigation.

Authors:  P S Olin; C R Lehner; J A Hilton
Journal:  Quintessence Int       Date:  1988-10       Impact factor: 1.677

Review 6.  The genetic and molecular basis of congenital eye defects.

Authors:  Jochen Graw
Journal:  Nat Rev Genet       Date:  2003-11       Impact factor: 53.242

Review 7.  Early support developmental journal for children with visual impairment: the case for a new developmental framework for early intervention.

Authors:  N Dale; A Salt
Journal:  Child Care Health Dev       Date:  2007-11       Impact factor: 2.508

Review 8.  Biology and therapy of inherited retinal degenerative disease: insights from mouse models.

Authors:  Shobi Veleri; Csilla H Lazar; Bo Chang; Paul A Sieving; Eyal Banin; Anand Swaroop
Journal:  Dis Model Mech       Date:  2015-02       Impact factor: 5.758

9.  HomozygosityMapper--an interactive approach to homozygosity mapping.

Authors:  Dominik Seelow; Markus Schuelke; Friedhelm Hildebrandt; Peter Nürnberg
Journal:  Nucleic Acids Res       Date:  2009-05-21       Impact factor: 16.971

10.  Mutation Detection in Patients with Retinal Dystrophies Using Targeted Next Generation Sequencing.

Authors:  Nicole Weisschuh; Anja K Mayer; Tim M Strom; Susanne Kohl; Nicola Glöckle; Max Schubach; Sten Andreasson; Antje Bernd; David G Birch; Christian P Hamel; John R Heckenlively; Samuel G Jacobson; Christina Kamme; Ulrich Kellner; Erdmute Kunstmann; Pietro Maffei; Charlotte M Reiff; Klaus Rohrschneider; Thomas Rosenberg; Günther Rudolph; Rita Vámos; Balázs Varsányi; Richard G Weleber; Bernd Wissinger
Journal:  PLoS One       Date:  2016-01-14       Impact factor: 3.240

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.