To date, at least 14 genes have been implicated in the development of ectopia lentis and its associated systemic conditions (including AASS, ADAMTS10, ADAMTS17, ADAMTSL4, ASPH, BCOR, CBS, COL18A1, FBN1, LTBP2, P3H2, PORCN, SUOX and VSX2). Here, ADAMTSL4 is linked to isolated ectopia lentis.