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A mucopolysaccharide is a long chain of sugars that is the building block of complex carbohydrates. Along with proteins and lipids, it forms a gelatinous material that is embedded between skin, bone, cartilage, and connective tissue cells. The polysaccharides provide lubrication, help transport substances between cells, and help hold together the cellular structure of connective tissue. They add to the structural integrity of bone, cartilage, skin, and other elastic membranes in the body. Defects in mucopolysaccharide production can result in or indicate a variety of conditions and diseases.
The thickness of body fluids, including blood, is also regulated, and as far as intercellular transport, the mucopolysaccharide helps in the exchange of nutrients and oxygen between blood vessels and cells. It also links with other proteins in the arterial walls, contributing to their strength. The healing of tissues back to their original integrity is also enabled by the high quantity found in wounds.
Mucopolysaccharide compounds are found at the cellular surface and in between cells. They are so essential to maintaining the structure of cells and for transporting nutrients that any deficiency can cause serious health consequences. A normal level in the blood keeps it flowing properly. Low levels of certain complex carbohydrates can thicken the blood and cause platelets to stick together more easily, forming blood clots.
If the enzymes that produce mucopolysaccharide molecules, also known as glycosaminoglycans, are missing or not working properly, various diseases, collectively known as mucopolysaccharidoses, result. Carbohydrates can build up in the cells. Depending on the disease, they can collect in the blood, connective tissue, skin, and the brain or other organs. The most severe forms of mucopolysaccharidosis (MPS) is MPS I, which is grouped into conditions such as Hurler, in which mental and physical development stops as soon as two years of age. Others include Hurler-Scheie and Scheie, while MPS II, known as Hunter syndrome, causes skeletal defects, lung problems, and skin lesions.
Mucopolysaccharide deficiencies can also result in Sanfilippo syndrome, Morquio syndrome, and Sly syndrome. Physical examination by a physician, urine tests, and enzyme assays are needed to determine what condition someone with symptoms of mucopolysaccharidoses has. These ailments can’t be cured, but medical treatments to alleviate symptoms include physical therapy, limiting of sugary and dairy products to lower mucus production, and surgery. Without enough complex carbohydrates produced the body cannot function properly, and genetic defects have lifelong consequences.
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