PhenomeGene — Human Disease Gene Website SeriesCPLANE1

Clinical Characteristics

Joubert syndrome (JBTS) is linked to problems with primary cilia proteins, affecting the development of various organs. Changes in these proteins can lead to brain malformation. JBTS shows developmental delay, muscle weakness, abnormal breathing patterns, abnormal eye movement, and a distinctive brain malformation seen on MRI called the "molar tooth sign."