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Pervasive genetic interactions modulate neurodevelopmental defects of the autism-associated 16p11.2 deletion in Drosophila melanogaster
Journal article   Open access   Peer reviewed

Pervasive genetic interactions modulate neurodevelopmental defects of the autism-associated 16p11.2 deletion in Drosophila melanogaster

Janani Iyer, Mayanglambam Dhruba Singh, Matthew Jensen, Payal Patel, Lucilla Pizzo, Emily Huber, Haley Koerselman, Alexis T Weiner, Paola Lepanto, Komal Vadodaria, …
Nature communications, Vol.9(1), 2548
06/29/2018
DOI: 10.1038/s41467-018-04882-6
PMCID: PMC6026208
PMID: 29959322
url
https://doi.org/10.1038/s41467-018-04882-6View
Published (Version of record) Open Access

Abstract

As opposed to syndromic CNVs caused by single genes, extensive phenotypic heterogeneity in variably-expressive CNVs complicates disease gene discovery and functional evaluation. Here, we propose a complex interaction model for pathogenicity of the autism-associated 16p11.2 deletion, where CNV genes interact with each other in conserved pathways to modulate expression of the phenotype. Using multiple quantitative methods in Drosophila RNAi lines, we identify a range of neurodevelopmental phenotypes for knockdown of individual 16p11.2 homologs in different tissues. We test 565 pairwise knockdowns in the developing eye, and identify 24 interactions between pairs of 16p11.2 homologs and 46 interactions between 16p11.2 homologs and neurodevelopmental genes that suppress or enhance cell proliferation phenotypes compared to one-hit knockdowns. These interactions within cell proliferation pathways are also enriched in a human brain-specific network, providing translational relevance in humans. Our study indicates a role for pervasive genetic interactions within CNVs towards cellular and developmental phenotypes.
Phenotype RNA, Small Interfering - genetics Sequence Deletion Autistic Disorder - pathology Cell Proliferation Autistic Disorder - metabolism Humans Male Drosophila Proteins - metabolism Gene Regulatory Networks Drosophila melanogaster - genetics DNA Copy Number Variations Brain - metabolism Drosophila melanogaster - metabolism Neurogenesis - genetics Gene Expression Regulation, Developmental Base Sequence Female Drosophila Proteins - antagonists & inhibitors Disease Models, Animal Autistic Disorder - genetics Chromosomes, Insect - chemistry Nerve Tissue Proteins - antagonists & inhibitors Nerve Tissue Proteins - genetics Sequence Homology, Nucleic Acid Protein Interaction Mapping Nerve Tissue Proteins - metabolism Animals Brain - pathology Chromosomes, Human, Pair 16 - chemistry Drosophila melanogaster - growth & development Drosophila Proteins - genetics RNA, Small Interfering - metabolism

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