Mucopolysaccharides are long chains of sugars that form complex carbohydrates, providing lubrication, transport, and structural integrity to connective tissue. Deficiencies can cause serious health consequences, including mucopolysaccharidoses, which cannot be cured but can be managed with medical treatments.
A mucopolysaccharide is a long chain of sugars that is the building block of complex carbohydrates. Together with proteins and lipids, it forms a gelatinous material that embeds itself between skin, bone, cartilage, and connective tissue cells. Polysaccharides provide lubrication, help transport substances between cells, and help hold the cellular structure of connective tissue together. They add to the structural integrity of bone, cartilage, skin, and other elastic membranes in the body. Defects in the production of mucopolysaccharides can cause or indicate a variety of conditions and diseases.
The thickness of body fluids, including blood, is also regulated, and in terms of intercellular transport, mucopolysaccharide helps in the exchange of nutrients and oxygen between blood vessels and cells. It also binds with other proteins in artery walls, contributing to their strength. The healing of tissues back to their original integrity is also enabled by the large amount found in wounds.
Mucopolysaccharide compounds are found on the cell surface and between cells. They are so essential for maintaining cell structure 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 make platelets stick together more easily, forming blood clots.
If the enzymes that produce mucopolysaccharide molecules, also known as glycosaminoglycans, are missing or malfunctioning, various diseases, collectively known as mucopolysaccharidoses, result. Carbohydrates can accumulate in cells. Depending on the disease, they can accumulate in the blood, connective tissue, skin, and brain or other organs. The most severe forms of mucopolysaccharidoses (MPS) are MPS I, which is grouped into conditions such as Hurler, in which mental and physical development arrest as early 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 lead to Sanfilippo syndrome, Morquio syndrome, and Sly syndrome. Physical exams by a doctor, urinalysis, and enzyme tests are needed to determine what condition someone with symptoms of mucopolysaccharidoses has. These ailments cannot be cured, but medical treatments to alleviate symptoms include physical therapy, limiting sugary and dairy products to decrease mucus production, and surgery. Without enough complex carbohydrates produced, the body cannot function properly, and genetic defects have lifelong consequences.
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