This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Metabolism of lipid, Subject name: Biochemistry
Lack of alpha-oxidation of fatty acids leads to 
Select and explain. 
A. Accumulation of phytanic acid, B. Formation of dicarboxylic acid, C. Formation of propionic acid, D. Oxidation of branched chain fatty acid
A: Accumulation of phytanic acid
Answer: A: Accumulation of phytanic acid 
Explanation: Refsum&;s Disease It is a metabolic error due to lack of alpha-hydroxylase (phytanic acid oxidase) so that alpha-oxidation does not occur and phytanic acid accumulates in the tissues. The patient presents with severe neurological symptoms, polyneuropathy, retinitis pigmentosa, nerve deafness and cerebellar ataxia. Regressions of symptoms are observed with restricted dietary intake of phytanic acid. Milk is a good source of phytanic acid, which may be avoided.Ref: DM Vasudevan - Textbook of Biochemistry, 6th edition, page no: 135