Glucosylceramide is accumulated in –
Correct Answer: Gaucher's disease
Description: Ans. is 'c' i.e., Gaucher's disease DiseaseEnzyme DeficiencyLipid AccumulatingClinical SymptomsTay-Sachs diseaseHexosaminidase ACer--Glc--Gal(NeuAc) -- Gal NacGM2 GangliosideMental retardation, blindness, muscular weaknessFabry's diseasea-GalactosidaseCer--Glc--Gal-- Gal GlobotriaosylceramideSkin rash, kidney failure (full symptoms only in males; X-!inked recessive)Metachromatic leukodystrophyArylsulfatase ACer--Gal-- OSO3 3-SulfogalactosylceramideMental retardation and psychologic disturbances in adults; demyelinationKrabbe's diseaseb-GalactosidaseCer-- GalMental retardation; myelin almost absentGaucher's diseaseb-GlucosidaseCer-- Gal GlucosylceramideEnlarged liver and spleen, erosion of long bones, mental retardation in infantsNiemann-Pick diseaseSphingomyelinaseCer-- P--choline SphingomyelinEnlarged liver and spleen , mental retardation; fatal in early lifeFarber's diseaseCeramidaseAcyl-- Sphingosine CeramideHoarseness, dermatitis, skeletal deformation, mental retardation; fatal in early life
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