Oligosaccharides are chains of sugar molecules used in the building of bones, cartilage, skin, tendons and many other tissues in the body. “Oligo” means a few and “saccharide” is a general term for the sugar part of the molecule. In the course of normal life there is a continuous recycling process of building new oligosaccharides and breaking down old ones. In the course of normal life this recycling process requires a series of biochemical tools called enzymes.
People with ML I lack the specific enzyme or are low in the enzyme called alpha-neuraminidase which breaks down large oligosaccharides and removing a substance called sialic acid. When alpha-neuraminidase is not present or low in activity it is unable to break down oligosaccharides therefore sialic acid builds up the body causing damage to various tissues and organs in the body.
