WMS is caused by highly penetrant pathogenic variants in fibrillin-1 (FBN1), that cause autosomal dominant WMS (WMS2, OMIM #608328), while pathogenic variants in three other genes, ADAMTS10 (WMS1, OMIM #277600), LTBP2 (WMS3, OMIM#614819) ADAMTS17 (WMS4, OMIM#613195) cause autosomal recessive WMS3–5. The gene discussed is ADAMTS17; the disease is Weill-Marchesani syndrome 2, dominant.