In Zellweger syndrome, which of the following is absent?
Zellweger syndrome is a rare disease characterized by the absence of functional peroxisomes. the long chain fatty acids (C26-C36) are not oxidized. They accumulate in tissues, paicularly in brain, liver and kidney. Hence the disorder is also known as cerebrohepatorenal syndrome. This condition is apparent at bih and is characterized by profound neurologic impairment, victims often dying within a year. Two closely related conditions are neonatal adrenoleukodystrophy and infantile Refsum disease. Zellweger syndrome and these two conditions represent a spectrum of overlapping features, with Zellweger syndrome being the most severe (many proteins affected) and infantile Refsum disease the least severe
ER
Golgi apparatus
Mitochondria
Peroxisomes
NEET 2019