sphingolipidoses

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sphingolipidoses

A group of inborn errors of sphingolipid metabolism in which lysosphingolipids accumulate, inhibiting protein kinase C activity in signal transduction, cell differentiation and in tumor promotion. See Ceramide lactoside lipidosis, Fabry's disease, Fucosidosis, Gaucher disease, Gangliosidosis, Globoid cell dystrophy, Krabbe's disease, Metachromatic leukodystrophy, Niemann-Pick disease, Sanhoff's disease, Tay-Sachs disease.

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leukodystrophy lysosomal storage disease sphingolipidosis storage disease			Sphingolipidoses are inherited lysosomal storage disorders caused by lysosomal hydrolase deficiency leading to storage of specific glycosphingolipids. HPLC for simultaneous quantification of total ceramide, ... by Groener, Johanna E.M.; Poorthuis, Ben J.H.M.; Kuiper, Sijmen; Helmond, Mariette T.J.; Hollak, Carla / Clinical Chemistry Gaucher disease: Understanding the molecular pathogenesis of sphingolipidoses. Orthopaedic manifestations of Gaucher disease by Lutsky, Kevin F.; Tejwani, Nirmal C. / Bulletin of the NYU Hospital for Joint Diseases			spherule spherules sphincter sphincter ani sphincter choledochus sphincter muscle of common bile duct sphincter muscle of pancreatic duct sphincter muscle of pupil sphincter muscle of pylorus sphincter muscle of urethra sphincter muscle of urinary bladder sphincter of Oddi sphincter of Oddi dysfunction sphincter pupillae sphincter spasm sphincter- sphincteralgia sphincterectomy sphincterismus sphincteritis sphincterolysis sphincteroplasty sphincterotomy sphinganine sphingolipid sphingolipidoses sphingolipidosis sphingolipodystrophy sphingomyelin sphingomyelin lipidosis sphingomyelin phosphodiesterase sphingomyelinosis sphingosine Sphinx cat sphygm(o)- sphygm- sphygmic sphygmic interval sphygmo- sphygmobolometer sphygmochronograph sphygmodynamometer sphygmogram sphygmograph sphygmoid sphygmomanometer sphygmometer sphygmoplethysmograph sphygmoscope sphygmoscopy sphygmotonometer			sphincteral sphincteralgia sphincterectomy sphincteric sphincteric sphincteric sphincterismus sphincteritis sphincterolysis sphincteroplasty sphincterotomy Sphincters Sphincters Sphincters Sphinctozoa Sphindidae sphinganine sphinges sphinges sphingid Sphingidae Sphingidae sphingids sphingine Sphingoidea sphingolipid sphingolipid sphingolipid activator protein sphingolipid activator protein sphingolipid activator protein sphingolipidoses sphingolipidosis sphingolipidosis Sphingolipids Sphingolipids sphingolipodystrophy sphingomyelin sphingomyelin sphingomyelin sphingomyelin sphingomyelin lipidosis sphingomyelin lipidosis sphingomyelin lipidosis sphingomyelin phosphodiesterase Sphingomyelin phosphodiesterase D sphingomyelin ratio sphingomyelin ratio sphingomyelin ratio Sphingomyelinase sphingomyelinosis Sphingomyelins Sphingomyelins Sphingomyelins sphingosine sphingosine sphingosine Sphingosine 1-phosphate Sphingosine kinase sphingosylphosphorylcholine sphinx sphinx

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