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Nuclear receptor TLX, a NR2 hepatocyte NF4-like receptor, has been shown to affect anterior brain differentiation, retinal development, and vision. It has been suggested that TLX targets genes for RAR beta2 and Pax2 during retinal development. Homozygous TLX mutant mice are viable at birth but exhibit reduced forebrain development and are more aggressive than wild-type mice. Mice with deleted TLX locus exhibit cerebral hypoplasia, blindness, and extreme aggression (fierce (frc) phenotype). TLX expression has been documented in mouse brain and eye. ESTs have been isolated from normal human brain libraries.
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