mukopolüsahhariidide

Mukopolüsahhariidide, also known as glycosaminoglycans (GAGs), are long, unbranched polysaccharides consisting of repeating disaccharide units. These disaccharides are typically composed of a uronic acid and an amino sugar. The most common uronic acids are glucuronic acid and iduronic acid, while the amino sugars are usually glucosamine orgalactosamine. The amino groups are often sulfated. This sulfation is a key characteristic that contributes to the highly negative charge of GAGs, which in turn influences their interactions with other molecules, particularly water and cations.

GAGs are a vital component of the extracellular matrix in animals, playing crucial roles in structural integrity and cell signaling. They are found in connective tissues, cartilage, bone, skin, and the vitreous humor of the eye. Their ability to bind large amounts of water makes them excellent lubricants and shock absorbers, essential for joint function and tissue hydration. Examples of individual GAGs include hyaluronic acid, chondroitin sulfate, dermatan sulfate, heparan sulfate, and keratan sulfate. Each type has a specific distribution and function within the body. For instance, hyaluronic acid is abundant in synovial fluid, providing lubrication to joints, while chondroitin sulfate is a major component of cartilage, contributing to its compressive strength. Disruptions in GAG synthesis or metabolism are associated with various genetic disorders known as mucopolysaccharidoses.