<prompt> Zellweger syndrome pathology is-Mitochondrial defectGlycoxisomal defectPeroxisomal defectNone of the above  <response>: Ans is c Peroxisomal defect Zellweger syndrome cerebrohepatorenal syndromeo It is one of three peroxisome biogenesis disorders which belong to the Zellweger spectrum of peroxisome biogenesis disorders PBD-ZSD. The other two disorders are neonatal adrenoleukodystrophy NALD, and infantile Refsum disease 3RD. Zellweger syndrome is the most severe of these three disorderso Zellweger syndrome is an autosomal recessive disorder caused by mutations in genes PEX that encode peroxins, proteins required for the normal assembly of peroxisomeso As a result of impaired peroxisome function, an individuals tissues and cells can accumulate very long chain fatty acids VLCFA and branched chain fatty acids BCFA that are normally degraded in peroxisomeso Zellweger syndrome is associated with impaired neuronal migration, neuronal positioning, and brain development. In addition, individuals with Zellweger syndrome can show a reduction in central nervous system CNS myelin particularly cerebral, which is referred to as hypomyelination. 