Approximately 50 different biochemical reactions occur entirely or partially within a peroxisome.
By definition, a peroxisome must contain catalase, which is an enzyme that breaks down hydrogen peroxide.
For example, in severe cases of ZS, the failure is nearly complete, while in IRD, there is some degree of peroxisome activity.
In peroxisomal single-enzyme disorders, the peroxisome is intact and functioning, but there is a defect in only one enzymatic process, with only one corresponding biochemical abnormality.
The peroxisome is involved in breaking down VLCFAs to lengths that the body can use or get rid of.
The range of disease abnormalities may be a result of a corresponding range of peroxisome failure.
To complement genetic approaches we are establishing a confocal based screen for small molecules that interfere with peroxisome biogenesis.