What is MPS IIIB?
The disease MPS IIIB, also known as Sanfilippo syndrome type IIIB, is an inherited disease. It is one of a group of eleven different genetic diseases known as the MPS disorders. The MPS disorders are all classified as lysosomal storage diseases. Other better-known lysosomal storage diseases that occur in humans include Tay-Sachs disease and Gaucher disease. The feature that unites lysosomal storage diseases is that they have abnormal lysosomal function. The lysosome is an important structure of virtually all cells in the body, and serves as the “garbage disposal” of the cell. In humans MPS IIIB is seen in approximately one out of 73,000 live births. The lysosome is essentially a “bag” within cells of the body, which is filled with special enzymes. The lysosome’s function is to disassemble large molecules of a cell that need to be recycled or disposed of. The way in which molecules are dissembled in the lysosomes involves a series of steps, something like an automobile assembly line, bu
