This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: AIIMS 2018, Subject name: Biochemistry
In a cerebrohepatorenal syndrome, which of the following accumulate in brain? 
Select and explain. 
A. Pyruvate, B. Sho-chain fatty acid, C. Very long-chain fatty acid, D. Acetyl CoA
C: Very long-chain fatty acid
Answer: C: Very long-chain fatty acid 
Explanation: Zellweger syndrome Cerebro-Hepato-Renal Syndrome. Impaired neuronal migration, hypomyelination, hepatomegaly, renal cysts Autosomal recessive Rare Absence of Peroxisomes in almost all tissues, peroxisomes are responsible of oxidation of very long chain fatty acid which contain more than 22 carbon Accumulation of polyenic acids in brain, with carbon > 22 Severe neurological symptoms Most patients die within 1st year of life.