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Retinoid-related orphan receptor beta (ROR beta), a NR1 thyroid hormone receptor, affects the development and functioning of the central nervous system, including sensory input integration and circadian timing. Disruption of ROR beta in mice results in juvenile ataxia, retinal degeneration, circadian activity abnormalities, and delayed onset of male fertility. ROR beta binds to the monomeric response elements containing the sequence AnnTAGGTCA as a monomer. An alternatively spliced isoform, ROR beta2, has been isolated from rats, and the expression of ROR beta2 is confined to the pineal gland and retina. ROR beta2 shares common DNA- and putative ligand-binding domains with wild-type ROR beta but is characterized by a different amino-terminal domain. ROR beta expression has been documented in various regions of the mouse brain. ESTs have been isolated from human tissue libraries, including cancerous human kidney and normal brain, pineal and eye.