Accumulation of sphingomyelin in phagocytic cells is feature of
Ans. is 'b' ie., Niemann pick disease First three options are types of Lysosomal storage disordersThe characteristic feature of lysosomal storage disorders is accumulation of specific macromolecules within cells that are normally metabolized by the cells. This is because of deficiency of one or other lysosomal enzymesSome important lysosomal storage disorders , the deficient enzymes and the stored macromolecule are listed below--Lysomal Storage DiseaseDeficient EnzymeStored Material* Fabry's ds*a- Galactosidase AGlobotriaosyl ceramide* Gaucher's ds*Acid beta - glucosidaseGlucosyl-ceramidase* Niemann - Pick ds*SphingomyelinaseSphingomyelin* Tay -Sach's ds*b - hexosaminidase AGM2 gangliosides* Sandhoff ds*b - hexosaminidase A and BGM2 gangliosides* Hurler's ds*a - L-iduronidaseDermatan sulfate Heparan sulfate* Hunter's ds*Iduronate sulfataseDermatan sulfate* Metachromatic leukodystrophy*Arylsulfatase -ACerebroside - sulfate* Krabbe's ds*GalactosylceramidaseGalactosylceramide Galactosyl sphingosine
Gauchers disease
Niemann pick ds
Tay sachs disease
Downs syndrome
Phospholipids, Glycolipids and their disorders