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Identification of a novel idiopathic congenital nystagmus‑causing missense mutation, p.G296C, in the FRMD7 gene.
- Source :
-
Molecular medicine reports [Mol Med Rep] 2018 Sep; Vol. 18 (3), pp. 2816-2822. Date of Electronic Publication: 2018 Jul 09. - Publication Year :
- 2018
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Abstract
- Exploring the genetic basis for idiopathic congenital nystagmus is critical for improving our understanding of its molecular pathogenesis. In the present study, direct sequencing using gene specific primers was performed in order to identify the causative mutations in two brothers from a Chinese family who had been diagnosed with idiopathic congenital nystagmus. A comprehensive ophthalmological examination, including eye movement recordings, fundus examination, and retinal optical coherence tomography imaging was also conducted, to characterize the disease phenotype. The results revealed that the two brothers exhibited clear signs of nystagmus without any other ocular anomalies. Direct sequencing revealed a G to T transition (c.886G>T) in exon 9 of the four‑point‑one, ezrin, radixin, moesin domain‑containing 7 (FRMD7) gene, which resulted in a conservative substitution of glycine to cysteine at codon 296 (p.G296C), leading to idiopathic congenital nystagmus in the two affected brothers. c.886G>T is a novel idiopathic congenital nystagmus‑inducing mutation in the FRMD7 gene. This finding expands the spectrum of known gene mutations in idiopathic congenital nystagmus, and may be useful for faster gene diagnosis, prenatal testing, the development of potential gene therapies, and for improving the understanding of the molecular pathogenesis of idiopathic congenital nystagmus.
- Subjects :
- Alleles
Amino Acid Sequence
Case-Control Studies
Child
Cytoskeletal Proteins chemistry
DNA Mutational Analysis
Exons
Humans
Male
Membrane Proteins chemistry
Mutation, Missense
Nystagmus, Congenital genetics
Pedigree
Phenotype
Cytoskeletal Proteins genetics
Membrane Proteins genetics
Nystagmus, Congenital diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 1791-3004
- Volume :
- 18
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Molecular medicine reports
- Publication Type :
- Academic Journal
- Accession number :
- 30015830
- Full Text :
- https://doi.org/10.3892/mmr.2018.9260