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In brain, one of the principal GSLs is sulfatide (SF), sulfated derivative of GalCer.

In brain, one of the principal GSLs is sulfatide (SF), sulfated derivative of GalCer. Altered SF level is implicated in Alzheimer’s and Parkinson’s diseases, multiple sclerosis, progressive epilepsy with mental retardation and other neurodegenerative human disorders. Goals:

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In brain, one of the principal GSLs is sulfatide (SF), sulfated derivative of GalCer.

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  1. In brain, one of the principal GSLs is sulfatide (SF), sulfated derivative of GalCer. Altered SF level is implicated in Alzheimer’s and Parkinson’s diseases, multiple sclerosis, progressive epilepsy with mental retardation and other neurodegenerative human disorders • Goals: • To determine how SO3 group affects SF anchoring to GLTP • To switch GLTP for SF selectivity 2-

  2. SF head group binding to GLTP K55I K55F K55Y K55Q K55E K55N

  3. “OUT” “IN” D48V WT

  4. PART III.Structural Basis for Suppression of Human Trinucleotide Repeat Expansion Diseases (TREDs) • repeated CNG-triplet sequences in genes • dynamically unstable, tend to expand • expanded tracts cause many muscle- and • neurodegenerative diseases

  5. H Y P O T H E S I S

  6. Ye, K., Malinina, L., and Patel, D.J. (2003) Recognition of small interfering RNA by a viral suppressor of RNA silencing. Nature 426, 874-878

  7. pGG(CAG)5CC pUUG(CUG)5CU • • • • • • • • • • • • • CC(GAC)5GGp UC(GUC)5GUUp A•A G•G U•U C•C

  8. 3? 1 2

  9. Sequence specific p19? 2 3 CNGCNGCNG • • • • • • • • • GNCGNCGNC

  10. Come to Bilbao Research Team Borja Ochoa Jevgenia Tamjar Felipe Goñi Elizaveta Katorcha Sergey Ivaninskiy Valeria Samygina Aintzane Cabo Sandra Delgado

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