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Ranbp1 , a Ran GTPase-binding protein implicated in nuclear/cytoplasmic trafficking, is included within the DiGeorge/22q11.2 Deletion Syndrome (22q11.2 DS) critical region associated with behavioral impairments including autism and schizophrenia.  Ranbp1  is highly expressed in the developing forebrain ventricular/subventricular zone but has no known obligate function during brain development. We assessed the role of  Ranbp1  in a targeted mouse mutant.  Ranbp1 −/−   mice are not recovered live at birth, and over 60% of  Ranbp1 −/−   embryos are exencephalic. Non-exencephalic  Ranbp1 −/−   embryos are microcephalic, and proliferation of cortical progenitors is altered. At E10.5, radial progenitors divide more slowly in the  Ranpb1  −/−  dorsal pallium. At E14.5, basal, but not apical/radial glial progenitors, are compromised in the cortex. In both E10.5 apical and E14.5 basal progenitors, M phase of the cell cycle appears selectively retarded by loss of  Ranpb1  function.  Ranbp1  −/− -dependent proliferative deficits substantially diminish the frequency of layer 2/3, but not layer 5/6 cortical projection neurons.  Ranbp1  −/−  cortical phenotypes parallel less severe alterations in  LgDel  mice that carry a deletion parallel to many (but not all) 22q11.2 DS patients. Thus,  Ranbp1  emerges as a microcephaly gene within the 22q11.2 deleted region that may contribute to altered cortical precursor proliferation and neurogenesis associated with broader 22q11.2 deletion.

cerebral cortex

Ranbp1 , Deleted in DiGeorge/22q11.2 Deletion Syndrome, is a Microcephaly Gene That Selectively Disrupts Layer 2/3 Cortical Projection Neuron Generation