Abstract

We studied the scientific literature and disease guidelines in order to summarize the clinical utility of the genetic test for cone rod dystrophies (CORDs). CORDs are caused by variations in the ABCA4, ADAM9, AIPL1, C8orf37, CACNA1F, CACNA2D4, CDHR1, CNGA3, CRX, DRAM2, GUCA1A, GUCY2D, HRG4, KCNV2, PDE6C, PITPNM3, POC1B, PROM1, PRPH2, RAB28, RAX2, RIMS1, RPGRIP1, RPGR SEMA4A, TTLL5 genes, with an overall prevalence of 1 per 40 000. Most genes have autosomal recessive inheritance; the others have autosomal dominant or X-linked recessive transmission. Clinical diagnosis is based on clinical findings, color vision testing, ophthalmological examination and electroretinography. The genetic test is useful for confirming diagnosis, and for differential diagnosis, couple risk assessment and access to clinical trials.

Details

Title
Genetic testing for cone rod dystrophies
Author
Abeshi, Andi 1 ; Zulian, Alessandra 2 ; Beccari, Tommaso 3 ; Dundar, Munis 4 ; Ziccardi, Lucia 5 ; Bertelli, Matteo 6 

 MAGI Balkans, Tirana, Albania; MAGI’S Lab, Rovereto, Italy 
 MAGI Euregio, Bolzano, Italy 
 Department of Pharmaceutical Sciences, University of Perugia, Perugia, Italy 
 Department of Medical Genetics, Erciyes University Medical School, Kayseri, Turkey 
 Neurophthalmology Unit, “G.B. Bietti” Foundation IRCCS, Rome, Italy 
 MAGI’S Lab, Rovereto, Italy; MAGI Euregio, Bolzano, Italy 
Pages
35-37
Publication year
2017
Publication date
2017
Publisher
De Gruyter Poland
e-ISSN
2564615X
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3156966115
Copyright
© 2017. This work is published under http://creativecommons.org/licenses/by-nc-nd/4.0 (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.