A neonatal baby boy is observed to have malformed hands, feet, and skull. Additional examination displays jaundice, hepatomegaly, and hypotonic muscles with psychomotor retardation. The pediatrician diagnoses the disorder as cerebrohepatorenal syndrome (Zellweger's syndrome). This condition is due to problems with which of the following organelles?

Question

Quizplus · Accepted Answer

Cerebrohepatorenal syndrome (Zellweger's syndrome) is a peroxisomal disorder that results from mutation of genes encoding for peroxins. This leads to the absence or malfunctioning of peroxisomes in the affected individual, which leads to the accumulation of very-long-chain fatty acids and other metabolic products, causing the symptoms observed in the neonatal baby boy.

A Neonatal Baby Boy Is Observed to Have Malformed Hands