This disease is caused by deficiency of an enzyme called alpha galactosidase A, which normally breaks down a fatty compound called lipid. Due to the fact that lipid is not broken down and excreted out of the body, one substance in that compound called globotriaosylceramide abnormally grows in number.
This compound accumulates and lines the walls of blood vessels, organs and other tissues. This narrows the passageway of blood and affects the supply of nutrients that travel throughout the body. Consequently when there is reduced blood flow, it can affect the heart, brain, kidney, eyes and other vital organs.