Lysosomes break down this unwanted matter via enzymes, highly specialized proteins essential for survival. Lysosomal disorders like mucopolysaccharidosis are triggered when a particular enzyme exists in too small an amount or is missing altogether. Mucopolysaccharidosis type I (MPS I) is a rare lysosomal storage disorder characterized by an abnormal build-up of various toxic materials, called glycosaminoglycans (GAGs) in the body's cells.

Understanding the Context

Mucopolysaccharidosis type I (MPS I) is a condition that affects many parts of the body. Explore symptoms, inheritance, genetics of this condition. What is mucopolysaccharidosis (MPS)? The mucopolysaccharidoses (MPSs) are a group of related lysosomal storage diseases.

Key Insights

Lysosomes are compartments in cells that break down molecules and remove waste products from cells. There are many types of mucopolysaccharidosis depending on which specific enzyme is missing. The types are classified by Roman numerals and sometimes also given a specific name (for example, Hurler syndrome and Hunter syndrome). Mucopolysaccharidosis refers to a group of inherited conditions in which the body is unable to properly breakdown mucopolysaccharides (long chains of sugar molecules that are found throughout the body).