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What is Which of the following enzyme is deficient in Niemann pick disease? and explain in detail?
Niemann-Pick disease (NPD) is a lipid storage disorder that results from the deficiency of a lysosomal enzyme, acid sphingomyelinase. The enzymatic defect results in pathologic accumulation of sphingomyelin (which is a ceramide phospholipid) and other lipids in the monocyte-macrophage system. Systemic involvement includes progressive lung disease, hepatosplenomegaly, sho stature, and pancytopenia. Ref: Clinical Studies in Medical Biochemistry Edited By Robe H. Glew, Miriam D. Rosenthal, 2006, Page 192; Lehninger Principles of Biochemistry, 4th Edition, Page 356