In a cerebrohepatorenal syndrome, which of the following accumulate in brain?
(A) Pyruvate (B) Sho-chain fatty acid (C) Very long-chain fatty acid (D) Acetyl CoA
(C) 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.