<prompt> The enzyme deficient in Galactosemia is:-SphingomyelinaseHexosaminidaseGalactose 1- phosphate uridyl transferaseGlucocerebrosidase  <response>: Most Common Galactosemia is Galactose 1- phosphate uridyl transferase GALT deficiency Galactose Diet- from milkmilk products Galactokinase Galactose - 1- Phosphate GALT UDP - Galactose Epimerase UDP- Glucose Deficiency of any of the enzyme in galactose metabolism will lead to galactosemia Galactokinase deficiency: Minor type galactosemia Substrate galactose gets accumulated Galactose later gets reduced to Galactitol which causes Oil Drop Cataract GALT Galactose - 1 - phosphate Uridyl transferase deficiency: More common Leads to Classical type galactosemia Substrate galactose - 1 - phosphate gets accumulated in Liver and Brain Leading to Jaundice Hepatomegaly Mental retardation Oil drop cataract also occurs here because galactose-1-phosphate when gets accumulated inhibits galactokinase which leads to galactose accumulation Sphingomyelinase, Hexosaminidase and Glucocerebrosidase are all deficient in Sphingolipidoses 