The protein encoded by this gene belongs to the claudin family of tight junction associated proteins and is a major component of central nervous system myelin that is necessary for normal CNS function,[9] hearing,[10] and spermatogenesis.[8] There is growing evidence that the protein determines the permeability between layers of myelin sheaths[11] and, with its expression highly regulated during development, may play an important role in cellular proliferation and migration. In addition, the protein is a candidate autoantigen in the development of autoimmune demyelinating disease.[7] Finally, experiments in Cldn11-null mice demonstrate that behavioral phenotypes in open field tests, as well as defects in sound lateralization, accompany changes in neurotransmitter levels in the amygdala/ventral hippocampus and auditory brainstem. This study reveals a molecular mechanism by which changes to myelin membrane properties in the absence of degenerative pathology, could lead to neuropsychiatric disease in humans.[12]
