Ans: a (Gaucher disease) Ref: Vasudevan and Sreekumari, 4th ed.Gaucher disease is due to lack of glucocerebroside and substance accumulating is glucosyl ceramide.Lysosomal storage disorders/ sphingolipidosis/ lipid storage diseases:Disease Enzyme deficientSubstance accumulating * Pompe diseasea glucosidase (acid maltase)glycogen* Gaucher diseaseb glucocerebrosidaseglucosyl ceramide (cerebroside)* Fabrya galactosidaseglobotriaosyl ceramide (ceramyl trihexoside)* Krabbeb galactosidasegalactosyl ceramide* Niemann-Pick diseaseSphingomyelinaseGM1 gangliosidosis Glycogen storage disordersEnzyme deficient* Type I Von GirkeGlucose 6 phosphatase* Type II Pompe diseasea glucosidase* Coris (Forbes)Debranching enzyme* AndersonBranching enzyme* Me ArdleMuscle phosphorylase* Type IV Her diseaseLiver phosphorylase