Krabbe’s disease is due to deficiency of-
Correct Answer: Beta galactocerebroside
Description: Ans. B. Beta galactocerebrosidase Examples of SphingolipidosesDiseaseEnzyme DeficiencyLipid AccumulatingClinical SymptomsTay-Sachs diseaseHexosaminidaseACer--Glc-Gal(NeuAc)-GalNAcGM2 GangliosideMental retardation, blindness, muscular weaknessFabry diseasea-GalactosidaseCer-Glc-Gal-GalGlobotriaosylceramideSkin rash, kidney failure (full symptoms only in males; X-linked recessive)Metachromatic leukodystrophyArylsulfatase ACer--Gal-OSO3 3- SulfogalactosylceramideMental retardation and psychologic disturbances in adults; demyelinationKrabbe diseaseb-GalactosidaseCer-Gal Galactosyl ceramideMental retardation; myelin almost absentGaucher diseaseb-GlucosidaseCer-Glc 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 diseaseCeramidaseAcyl--Sphingosine CeramideHoarseness, dermatitis, skeletal deformation, mental retardation; fatal in early life
Category:
Biochemistry
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