Abstract

We studied the scientific literature and disease guidelines in order to summarize the clinical utility of genetic testing for ocular albinism and oculocutaneous albinism. Ocular albinism has X-linked recessive inheritance, with a prevalence that varies from 1/40000 to 1/1000000, and is caused by mutations in the GPR143 and CACNA1F genes. Oculocutaneous albinism has autosomal recessive inheritance, with an overall prevalence of 1/17000, and is caused by mutations in the TYR, OCA2, TYRP1, SLC45A2, SLC24A5 and C10orf11 genes. Clinical diagnosis involves ophthalmological examination, testing of visually evoked potentials (VEP) and electrophysiological testing (ERG). The genetic test is useful for confirming diagnosis, differential diagnosis, for couple risk assessment and access to clinical trials.

Details

Title
Genetic testing for ocular albinism and oculocutaneous albinism
Author
Abeshi, Andi 1 ; Marinelli, Carla 2 ; Beccari, Tommaso 3 ; Dundar, Munis 4 ; Falsini, Benedetto 5 ; Bertelli, Matteo 6 

 MAGI Balkans, Tirana, Albania; MAGI’S Lab, Rovereto, Italy 
 MAGI’S Lab, Rovereto, Italy 
 Department of Pharmaceutical Sciences, University of Perugia, Perugia, Italy 
 Department of Medical Genetics, Erciyes University Medical School, Kayseri, Turkey 
 Department of Ophthalmology, Catholic University of Rome, Rome, Italy 
 MAGI’S Lab, Rovereto, Italy; MAGI Euregio, Bolzano, Italy 
Pages
80-82
Publication year
2017
Publication date
2017
Publisher
De Gruyter Poland
e-ISSN
2564615X
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3156966184
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.