I-cell disease

I-cell disease A rare, severe genetic disorder of the lysosomes (organelles within a cell that contain enzymes that can lyse, or digest, substances). Multiple enzyme deficiencies associated with I-cell disease lead to the accumulation of certain fatty substances (mucolipids) and certain complex carbohydrates (mucopolysaccharides) within the cells of many tissues of the body. The name comes from the deposits of accumulated material, known as inclusion bodies. I-cell disease affects the kidneys, heart, and nervous system. I-cell disease is inherited in an autosomal recessive manner. There is no known treatment for I-cell disease. Also known as mucolipidosis II.