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Sphingolipid Disorders

S. Sphingolipid Disorders. Eric Niederhoffer SIU-SOM. Sphingolipids (phospho- or glycolipids). General Structure. Cer. PC. Sm. GlcCb (neutral GL). LacCer (Gb). Gan. neuraminidase (sialidase). NANA. NANA. b -galactosidase. b. b. b. b. Gal. GalNAc. Gal. Cer. Glu. NANA. b.

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Sphingolipid Disorders

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  1. S Sphingolipid Disorders Eric Niederhoffer SIU-SOM Sphingolipids (phospho- or glycolipids) General Structure Cer PC Sm GlcCb (neutral GL) LacCer (Gb) Gan

  2. neuraminidase (sialidase) NANA NANA b-galactosidase b b b b Gal GalNAc Gal Cer Glu NANA b b b b Cer Glu Gal GalNAc Gal NANA b-hexosaminidase A GM2 activator b b b Cer Glu Gal GalNAc NANA neuraminidase (sialidase) SAP-B b b Cer Glu Gal a-galactosidase A SAP-B b b b b a Cer Glu Gal Cer Glu Gal Gal b-galactosidase SAP-B, SAP-C b Cer Glu b-hexosaminidase A&B b b b a Cer Glu Gal Gal GalNAc b-glucosylceramidase SAP-C b-galactosylceramidase SAP-A, SAP-C b Cer PC Cer Cer Gal arylsulfatase A SAP-B sphingomyelinase b Cer Gal SO3H S + FA Gangliosidoses Generalized gangliosidosis GD1 GM1 Tay-Sachs disease GM2 Sialidosis GM3 Fabry’s disease Sandhoff’s disease Gaucher’s disease Krabbe’s disease Cerezyme Metachromatic leukodystrophy Niemann-Pick disease

  3. Targeting of Lysosomal Enzymes to Lysosomes Addition of M6P to lysosomal enzymes Recognition by MPRs M6P independent pathways

  4. Review Questions • How do you interpret ganglioside names (G, D, M, 1, 2, 3)? • What do the different lysosomal enzyme names mean in the context of removing saccharides? • Where does ganglioside degradation occur?

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