TY - JOUR
T1 - Genome-wide copy number variation in epilepsy
T2 - novel susceptibility loci in idiopathic generalized and focal epilepsies.
AU - Mefford, Heather C.
AU - Muhle, Hiltrud
AU - Ostertag, Philipp
AU - von Spiczak, Sarah
AU - Buysse, Karen
AU - Baker, Carl
AU - Franke, Andre
AU - Malafosse, Alain
AU - Genton, Pierre
AU - Thomas, Pierre
AU - Gurnett, Christina A.
AU - Schreiber, Stefan
AU - Bassuk, Alexander G.
AU - Guipponi, Michel
AU - Stephani, Ulrich
AU - Helbig, Ingo
AU - Eichler, Evan E.
PY - 2010
Y1 - 2010
N2 - Epilepsy is one of the most common neurological disorders in humans with a prevalence of 1% and a lifetime incidence of 3%. Several genes have been identified in rare autosomal dominant and severe sporadic forms of epilepsy, but the genetic cause is unknown in the vast majority of cases. Copy number variants (CNVs) are known to play an important role in the genetic etiology of many neurodevelopmental disorders, including intellectual disability (ID), autism, and schizophrenia. Genome-wide studies of copy number variation in epilepsy have not been performed. We have applied whole-genome oligonucleotide array comparative genomic hybridization to a cohort of 517 individuals with various idiopathic, non-lesional epilepsies. We detected one or more rare genic CNVs in 8.9% of affected individuals that are not present in 2,493 controls; five individuals had two rare CNVs. We identified CNVs in genes previously implicated in other neurodevelopmental disorders, including two deletions in AUTS2 and one deletion in CNTNAP2. Therefore, our findings indicate that rare CNVs are likely to contribute to a broad range of generalized and focal epilepsies. In addition, we find that 2.9% of patients carry deletions at 15q11.2, 15q13.3, or 16p13.11, genomic hotspots previously associated with ID, autism, or schizophrenia. In summary, our findings suggest common etiological factors for seemingly diverse diseases such as ID, autism, schizophrenia, and epilepsy.
AB - Epilepsy is one of the most common neurological disorders in humans with a prevalence of 1% and a lifetime incidence of 3%. Several genes have been identified in rare autosomal dominant and severe sporadic forms of epilepsy, but the genetic cause is unknown in the vast majority of cases. Copy number variants (CNVs) are known to play an important role in the genetic etiology of many neurodevelopmental disorders, including intellectual disability (ID), autism, and schizophrenia. Genome-wide studies of copy number variation in epilepsy have not been performed. We have applied whole-genome oligonucleotide array comparative genomic hybridization to a cohort of 517 individuals with various idiopathic, non-lesional epilepsies. We detected one or more rare genic CNVs in 8.9% of affected individuals that are not present in 2,493 controls; five individuals had two rare CNVs. We identified CNVs in genes previously implicated in other neurodevelopmental disorders, including two deletions in AUTS2 and one deletion in CNTNAP2. Therefore, our findings indicate that rare CNVs are likely to contribute to a broad range of generalized and focal epilepsies. In addition, we find that 2.9% of patients carry deletions at 15q11.2, 15q13.3, or 16p13.11, genomic hotspots previously associated with ID, autism, or schizophrenia. In summary, our findings suggest common etiological factors for seemingly diverse diseases such as ID, autism, schizophrenia, and epilepsy.
UR - http://www.scopus.com/inward/record.url?scp=77956628767&partnerID=8YFLogxK
U2 - 10.1371/journal.pgen.1000962
DO - 10.1371/journal.pgen.1000962
M3 - Article
C2 - 20502679
AN - SCOPUS:77956628767
SN - 1553-7390
VL - 6
SP - e1000962
JO - PLoS genetics
JF - PLoS genetics
IS - 5
ER -