Two genetic diseases, Hunter and Hurler syndromes, are caused by an inability of cells to break down and recycle mucopolysaccharides, which are substances found in the extracellular areas of the body. Which organelle is responsible for performing this function in normal cells
Answer: Lysosomes serve as the cell's garbage disposers, breaking down unwanted materials.
Explanation: Hurler syndrome is the most severe form of mucopolysaccharidosis type 1 (MPS1; see this term), a rare lysosomal storage disease, characterized by skeletal abnormalities, cognitive impairment, heart disease, respiratory problems, enlarged liver and spleen, characteristic facies and reduced life expectancy.
Hunter syndrome is a very rare, inherited genetic disorder caused by a missing or malfunctioning enzyme. In Hunter syndrome, the body doesn't have enough of the enzyme iduronate 2-sulfatase. This enzyme's job is to break down certain complex molecules, and without enough of this enzyme, the molecules build up in harmful amounts.
The buildup of massive amounts of these harmful substances eventually causes permanent, progressive damage affecting appearance, mental development, organ function and physical abilities.
Hunter syndrome is far more common in boys. The condition is one type of a group of inherited metabolic disorders called mucopolysaccharidoses (MPSs). Hunter syndrome is also known as MPS II.
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