"in the connective tissue of animals" or mammals? Is dermatan sulfate what causes myxomatous degeneration?
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Dermatan sulfate is a carbohydrate found in the connective tissue of animals. A polysaccharide, it forms into long chains made of repeating molecular units. These chains are a major structural component of the material that fills the space between cells throughout the body; their role is similar to that of cellulose and other forms of fiber in plants. Dermatan sulfate is found especially in the skin, where it is the most common molecule of its kind, and in the walls of blood vessels.
Unfortunately, it's impossible to discuss the chemical nature of this substance without using a lot of very long words. It is classified as a glycosaminoglycan (GAG), a group of chemicals also called mucopolysaccharides. These are linear carbohydrate chains made of repeating subunits; each subunit is a molecule made of two simple sugars. In dermatan sulfate, these two sugars are iduronic acid (IdUA) and n-acetylgalactosamine (GalNAc). Other GAGs include keratan sulfate, chondroitin sulfate, and heparin.
Dermatan sufate is also known as beta-Heparin and is sometimes called chondroitin sulfate B. It was once believed to be an alternate form of chondroitin sulfate, a similar molecule that is the most common GAG and also a major component of cartilage. They are now considered to be different chemicals. The chondroitin sulfate molecule contains glucuronic acid (GlcUA) in place of iduronic acid.
In addition to its role as a major constituent of the skin and other organs, dermatan sulfate is believed to play a part in repairing wounds, regulating the coagulation of blood, and responding to infections, though its role in these processes is not well understood. Along with heparin and a few other chemicals in the same family, it is sometimes injected as an anticoagulant drug.
Dermatan sulfate is also thought to play a role in a number of diseases, including tumor formation, fibrosis, and various developmental syndromes. Glycosaminoglycans are generally not water-soluble and require special enzymes to be broken down. Occasionally, individuals have trouble producing these enzymes, and GAGs can build up in the body.
Diseases where this occurs are called mucopolysaccharidosis disorders. Excesses of dermatan sulfate have been linked to bone lesions, corneal opacity and blindness, and physical and mental developmental disorders. Mucopolysaccharidosis disorders can sometimes be treated with bone marrow transplants or enzyme replacement therapy.
When dermatan sulfate builds up in the mitral valve of the heart, it can lead to mitral valve prolapse. It is also believed to play a key role in atherosclerosis, the buildup of plaque in the arterial walls. Investigation continues into what role it may play in other diseases of the heart and cardiovascular system.
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