Zellweger syndrome pathology is-

Ans is 'c' Peroxisomal defect Zellweger syndrome (cerebrohepatorenal syndrome)o 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 individual's 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.