PhenomeGene — Human Disease Gene Website SeriesCPLANE1

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Joubert syndrome (JBTS), first described in 1969, is a rare lethal congenital disorder. CPLANE1was first reported as a causative gene of JBTS in a portion of families. The presence of truncated variants in CPLANE1is associated with oral-facial-digital syndrome type VI and results in a severe phenotype and early death.