Question

What is a Peroxisomal Disorder?

Answer 1

Peroxisomal Disorder:

1. Peroxisomes are vesicles, single membrane-bound organelles found in the eukaryotes.
2. These perform oxidation reactions using molecular oxygen and release hydrogen peroxide.
3. Peroxisomes also contain catalase enzyme for decomposition of hydrogen peroxide which is harmful to cells.
4. These organelles contain about 50 important digestive enzymes for breaking down toxic materials in the cell and oxidative enzymes for metabolic activity.
5. Liver cells contain most of the peroxisomes.
6. Peroxisomal disorders are a group of disorders that are caused by the dysfunction of peroxisomes.
7. The disease is referred to as peroxisome biogenesis disorders (PBDs)
8. It is caused by mutations in one of 13 PEX genes.
9. The common mutation in PEX1 gene.
10. Examples: Refsum disease, zellweger syndrome, X-linked adrenoleukodystrophy