Amish, Mennonite, and Hutterite
Genetic Disorder Database

Familial focal epilepsy with variable foci 3

Disorder
OMIM #: #617118  (Click to access OMIM database)
Disorder: Familial focal epilepsy with variable foci 3 
Also known as: Familial focal epilepsy and focal cortical dysplasia 
Clinical
Phenotype: focal cortical dysplasia, focal seizures 
Seen In: Amish
Old Order Mennonite
Old Colony Mennonite
Unknown/Other Mennonite
Hutterite
Remarks: Autosomal dominant 
Mutations
1   Old Order Mennonite  
Gene: NPRL3
Base Change: c.349delG
Amino Acid Change: p.Glu117Lysfs*5
Last updated: 2022-11-04 

References
Iffland PH, Everett ME, Cobb-Pitstick KM, Bowser LE, Barnes AE, Babus JK, Romanowski AJ, Baybis M, Elziny S, Puffenberger EG, Gonzaga-Jauregui C, Poulopoulos A, Carson VJ, Crino PB. (2022) NPRL3 loss alters neuronal morphology, mTOR localization, cortical lamination, and seizure threshold. Brain Feb 8:awac044.
PubMed ID: 35136953 
Strauss KA, Gonzaga-Jauregui C, Brigatti KW, Williams KB, King AK, Van Hout C, Robinson DL, Young M, Praveen K, Heaps AD, Kuebler M, Baras A, Reid JG, Overton JD, Dewey FE, Jinks RN, Finnegan I, Mellis SJ, Shuldiner AR, Puffenberger EG. (2018) Genomic diagnostics within a medically underserved population: efficacy and implications. Genet Med Jan;20(1):31-41.
PubMed ID: 28726809 

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