It is not usually present in the body in measurable amounts. Oligodendrocytes, the myelin synthesizing cells in the central nervous system, are killed when psychosine is not degraded as usual. There are specialized proteins in the body, called enzymes, which are responsible for the breakdown of carbohydrates, fats and other proteins. Enzyme activity is a measure of how well the enzyme does its job. People with an enzyme deficiency have little or no enzyme activity.
Lysosomes are substructures of the cell where many enzymes are stored and do their work.
Lysosomes are the sites where the basic elements of degraded lipids, proteins and starches are recycled. It is responsible for the breakdown of cerebroside and psychosine. GALC is the enzyme deficient in individuals with Krabbe disease. If GALC does not work correctly, cerebroside and psychosine will not be degraded as usual. The presence of globoid cells is a critical diagnostic feature of this condition, which is also known as globoid cell Leukodystrophy.