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What is Which organelle is involved in the case of sphinghomyelin deficiency: and explain in detail?
Ans. (a) LysosomeRef: Harper's Biochemistry, 30th ed. pg. 251* Sphinghomyelin is a major neural tissue component, the core structure of which is formed by ceramide.* It is the only sphingholipid without sugar.* The common sphinghomyelin deficiency is Neiman-Pick disease. The organelle which is involved is the lysosomes which allow accumulation of sphinghomyelin.* Signs & symptoms of Niemen pick's: Hepatosplenomegaly, decreased appetite, unsteady gait, ataxia, slurring of speech (due to accumulation of sphingholipids in neural tissue).