<prompt> A child presents with hypotonia and seizures. It was confirmed to be cerebrohepatorenal syndrome. Which of the following is accumulated in the brain in cerebrohepatorenal syndrome?GlucoseLong chain fatty acidTriglyceridesLactic acid  <response>: Cerebrohepatorenal syndrome or Zellwegers syndrome is characterised by abnormalities in the synthesis of bile acids and a marked reduction of plasmalogens. Long-chain fatty acids are accumulated in Zellwegers syndrome. It is a rare inherited absence of peroxisomes. It is due to mutations in genes encoding proteins called peroxins, involved in peroxisome biogenesis. Such individuals accumulate C26-C38 polyenoic acids in brain tissues and also exhibit a generalised loss of peroxisomal functions. It causes severe neurological symptoms and most patients die in first year of life. Treatment is often suppoive and symptom-based. Reference: Harpers Illustrated Biochemistry 31st edition Page nos 215 