PUBLICATION

Common DISC1 Polymorphisms Disrupt Wnt/GSK3β Signaling and Brain Development

Authors
Singh, K.K., De Rienzo, G., Drane, L., Mao, Y., Flood, Z., Madison, J., Ferreira, M., Bergen, S., King, C., Sklar, P., Sive, H., and Tsai, L.H.
ID
ZDB-PUB-111122-37
Date
2011
Source
Neuron   72(4): 545-558 (Journal)
Registered Authors
De Rienzo, Gianluca, Sive, Hazel
Keywords
none
MeSH Terms
  • Animals
  • Brain/growth & development*
  • Brain Chemistry/genetics*
  • Cell Line, Tumor
  • Female
  • Genetic Variation/genetics
  • Glycogen Synthase Kinase 3/genetics*
  • HEK293 Cells
  • Humans
  • Mice
  • Nerve Tissue Proteins/genetics*
  • Phenotype
  • Polymorphism, Genetic/genetics*
  • Pregnancy
  • Signal Transduction/genetics*
  • Wnt3A Protein/genetics*
  • Zebrafish
PubMed
22099458 Full text @ Neuron
Abstract
Disrupted in Schizophrenia-1 (DISC1) is a candidate gene for psychiatric disorders and has many roles during brain development. Common DISC1 polymorphisms (variants) are associated with neuropsychiatric phenotypes including altered cognition, brain structure, and function; however, it is unknown how this occurs. Here, we demonstrate using mouse, zebrafish, and human model systems that DISC1 variants are loss of function in Wnt/GSK3β signaling and disrupt brain development. The DISC1 variants A83V, R264Q, and L607F, but not S704C, do not activate Wnt signaling compared with wild-type DISC1 resulting in decreased neural progenitor proliferation. In zebrafish, R264Q and L607F could not rescue DISC1 knockdown-mediated aberrant brain development. Furthermore, human lymphoblast cell lines endogenously expressing R264Q displayed impaired Wnt signaling. Interestingly, S704C inhibited the migration of neurons in the developing neocortex. Our data demonstrate DISC1 variants impair Wnt signaling and brain development and elucidate a possible mechanism for their role in neuropsychiatric phenotypes.
Genes / Markers
Figures
Show all Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping