<prompt> Deficiency of the enzyme phytate oxidase results in?Zillwegers diseaseLows syndromeRefsums diseaseTay-sachs disease  <response>: Ans. c Refsums disease. Ref. Harper, Biochemistry, 25th ed., 248INBORN ERRORFEATURERefsums diseaseIt is a rare inherited disorder caused by accumulation of phytanic acidIts due to the deficiency of phytate oxidase and defect in alpha oxidation.It basically leads to peripheral neuropathy due to accumulation of phytanic acid in nerves,and retinitis pigmentosa is other associated feature.Zellwegers diseasecerebrohepatorenal syndrome that occurs due to inherited absence of peroxisomes.Lowes syndromeis occulocerebrorenal syndrome. Heart is not involvedTay-sachs diseaseis a sphingolipidosis Hexosaminidase A is absent 