Which organelle is involved in the case of sphinghomyelin deficiency:: (A) Lysosome, (B) Nucleus, (C) Mitochondria, (D) Cell membrane
Answer is A. 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).