Reelin
Reelin is a large secreted extracellular matrix glycoprotein that helps regulate processes of neuronal migration and positioning in the developing brain by controlling cell-cell interactions.
Function
Reelin is crucial for the correct positioning of neurons in the brain. It is secreted by Cajal-Retzius cells in the marginal zone of the developing cortex and hippocampus, and by granule cells in the olfactory bulb. Reelin binds to very-low-density lipoprotein receptors (VLDLR) and apolipoprotein E receptor 2 (ApoER2), initiating a signaling cascade that includes the activation of the intracellular adapter protein Disabled-1 (Dab1).
Clinical significance
Mutations in the Reelin gene or in its downstream signaling partners result in a characteristic "reeler" phenotype in mice, characterized by a disorganized cortical structure. In humans, these mutations are associated with lissencephaly, a severe form of brain malformation.
Reelin has also been implicated in several psychiatric disorders. Reduced levels of Reelin have been found in the brains of patients with schizophrenia, bipolar disorder, and major depression. It is thought that this reduction may contribute to the cognitive deficits observed in these disorders.
See also
References
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Contributors: Prab R. Tumpati, MD
