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SUMMARY:BMI SEMINAR // Vittorio Maglione - Sphingolipid metabolism: new pe
 rspectives for treating Huntington's  disease
DTSTART:20190116T121500
DTEND:20190116T131500
DTSTAMP:20260427T215021Z
UID:47d992314a6551a22b2947ce3f7170d76ce4a0fb3af2fdff92c1c52f
CATEGORIES:Conferences - Seminars
DESCRIPTION:Vittorio Maglione\, Centre for Neurogenetics and Rare Diseases
 \, IRCCS Neuromed\, Pozzilli (IS)\, Italy\nHuntington's disease (HD)\, the
  most common dominantly inherited neurodegenerative disorder is characteri
 zed by a progressive striatal and cortical neurodegeneration associated wi
 th cognitive and behavioral disturbance.\nSo far\, many are the aberrant m
 olecular mechanisms described to be associated with the disease\, however 
 much remains to be defined. In the last decade\, perturbed metabolism of g
 angliosides\, glycosphingolipids most abundant in the brain\, has been des
 cribed to play an essential role in the pathogenesis of the disease. In th
 is context\, we have contributed to demonstrate that metabolism of GM1 gan
 glioside is impaired in HD and its modulation results therapeutically effe
 ctive in different HD pre-clinical models.\nMore recently\, our research h
 as revealed that sphingolipid breakdown is not restricted to ganglioside m
 etabolism\, but it also affects regulation of Sphingosine-1-phosphate (S1P
 )\, a potent signaling lipid that regulates a number of processes essentia
 l to cellular homeostasis\, and viability. S1P metabolism is significantly
  disrupted in HD\, even at early stage of the disease and\, importantly\, 
 modulation of its metabolism/axis results beneficial in multiple HD pre-cl
 inical models.\nTo date\, there exists a variety of molecules/drugs that s
 electively target S1P metabolism/axis\, currently used in clinical trials 
 for human disorders. Progress in the understanding of S1P biology in HD wo
 uld definitely enhance therapeutic perspectives for the treatment of the d
 isease\, by taking advantage from the already available molecules and by p
 romoting the development of new ones.\n 
LOCATION:SV 1717 https://plan.epfl.ch/?room==SV%201717
STATUS:CONFIRMED
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