This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Lipids, Subject name: Biochemistry
What is the biochemical defect in the Zellweger syndrome? 
Select and explain. 
A. Peroxisomal biogenesis disorder, B. Lysosomal targeting disorder, C. Defect in glycosylation of proteins, D. Trisomy 21
A: Peroxisomal biogenesis disorder
Answer: A: Peroxisomal biogenesis disorder 
Explanation: Ans. A. Peroxisomal biogenesis disorderBiochemical defect in Zellweger SyndromeThere is gene defects, involving mainly the import of proteins that contain the PTS1 targeting signal* Zellweger syndrome (most severe)Clinical picture* Typical facial appearance (high forehead, unslanting palpebral fissures, hypoplastic supraorbital ridges, and epicanthal folds)* Severe weakness and hypotonia, neonatal seizures* Eye abnormalities (cataracts, glaucoma, corneal clouding, Brushfield spots, pigmentary retinopathy, and nerve dysplasia)* Because of the hypotonia and "mongoloid" appearance, Down syndrome may be suspected