Abstract

In this report, we present a European family with six individuals affected with Moyamoya disease (MMD). We detected two novel missense variants in the Moyamoya susceptibility gene RNF213, c.12553A>G (p.(Lys4185Glu)) and c.12562G>A (p.(Ala4188Thr)). Cosegregation of the variants with MMD, as well as a previous report of a variant affecting the same amino acid residue in unrelated MMD patients, supports the role of RNF213 in the pathogenesis of MMD.

Details

Title
Novel missense variants in the RNF213 gene from a European family with Moyamoya disease
Author
Gagunashvili, Andrey N 1 ; Ocaka Louise 1 ; Kelberman, Daniel 1 ; Munot Pinki 2 ; Bacchelli Chiara 1 ; Beales, Philip L 1 ; Ganesan Vijeya 3 

 UCL Great Ormond Street Institute of Child Health, GOSgene, Genetics and Genomic Medicine, London, UK (GRID:grid.83440.3b) (ISNI:0000000121901201) 
 Great Ormond Street Hospital for Children NHS Foundation Trust, Neurology Department, London, UK (GRID:grid.424537.3) (ISNI:0000 0004 5902 9895) 
 Great Ormond Street Hospital for Children NHS Foundation Trust, Neurology Department, London, UK (GRID:grid.424537.3) (ISNI:0000 0004 5902 9895) ; UCL Great Ormond Street Institute of Child Health, Clinical Neurosciences, London, UK (GRID:grid.83440.3b) (ISNI:0000000121901201) 
Publication year
2019
Publication date
Aug 2019
Publisher
Springer Nature B.V.
e-ISSN
2054345X
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
2269410039
Copyright
This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.