Zellweger Syndrome

Zellweger Syndrome
Zellweger Syndrome is a member of the family of leukodystrophies. It is a peroxisomal disorder that appears most commonly as early developmental defects. Craniofacial abnormalities and liver dysfunction are common. It is a disorder that occurs when peroxisomes of the body do not function normally. It has a negative effect on the body's myelin, causing a reduction in myelination throughout the affected individual's Central Nervous System.

Hypomyelination
Plasmalogen is one of the substances produced by cellular Peroxisomes. Since plasmalogen is a necessary factor in the process of myelination, the disruption of peroxisomal function will disrupt the myelination of axons in the body.

Prognosis and Severity
In the family of leukodystrophies, Zellweger syndrome is considered among the most severe. There is no cure for Zellweger syndrome, and affected newborns are unlikely to achieve a lifespan past infancy or one year of age. This can be attributed to the fact that Zellweger syndrome causes infantile apnea and difficulty in feeding the newborn, as well as the previously mentioned defect in myelin production that reduces or erradicates the proper function of the developing central nervous system.

This is a Peroxisome.