G protein-coupled receptor-dependent development of human frontal cortex.

John Rubenstein

John Rubenstein

John Rubenstein

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This article reports the identification of an orphan G-protein coupled receptor (GPR56) as the abnormal gene in humans with bilateral frontoparietal polymicrogyria (BFPP), a regional defect in cerebral cortical development. This is the first example of GPCR-signaling in the control of regional development of human cerebral cortex, and opens the door towards identifying additional ligands that participate in regional development of the cortex.

Silvia Arber

Silvia Arber

Silvia Arber

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This paper identifies the previously cloned orphan G-protein coupled receptor GPR56 by linkage analysis from a human disease causing cortex malformations. Interestingly, not all cortical areas are equally affected in this disease and the study therefore suggests GPR56 may be a candidate gene which could be involved in differential patterning of frontal cortical regions. Moreover, it shows that G-protein coupled receptors may be part of important signaling pathways involved in cortex development.