All WS disease mutations described thus far are heterozygous nucleotide substitutions occurring at the splice acceptor of intron 7 or splice donor site of intron 8 of the versican (VCAN) gene, also known as chondroitin sulfate proteoglycan-2 (CSPG2), leading to aberrant splice products and/or to an imbalanced quantitative ratio of the four VCAN transcript variants [2-13]. Here, VCAN is linked to Werner syndrome.