Mutations in OCRL1 that encodes for inositol-5-phosphatase, another Rab8 effector cause the X-linked oculocerebrorenal syndrome of Lowe (OCRL) or Lowe syndrome which manifests with abnormal primary cilia (Hou et al., 2011; Coon et al., 2012). Here, RAB8A is linked to oculocerebrorenal syndrome.