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SUMMARY:Sirtuin1 prevents axonal degeneration in adreno-leukodystrophy
DTSTART:20140409T140000
DTEND:20140409T150000
DTSTAMP:20260930T185342Z
UID:b17d03759372e7861351a3574b133859186794198435cac1ea04dec0
CATEGORIES:Conferences - Seminars
DESCRIPTION:Aurora Pujol\, MD\, PhD\, Institut de Investigacio Biomedica d
 e Bellvitge (IDIBELL)\, Barcelona (E)\nSEMINAR of the LAUSANNE INTEGRATIVE
  METABOLISM and NUTRITION ALLIANCE (LIMNA)Abstract:\nOxidative stress and 
 mitochondria dysfunction are noxious factors\, intertwined in the ethiolog
 ical core of most common neurodegenerative conditions. In the peroxisomal 
 disease adrenoleukodystrophy\, caused by loss of function of the peroxisom
 al transporter of very long-chain fatty acids Abcd1\, we have identified a
 n early oxidative stress leading to late onset axonal degeneration and dis
 ability. In this study\, we demonstrate that excess of the VLCFA C26:0 gen
 erates mitochondrial ROS\, resulting in mitochondrial DNA and protein oxid
 ation. This is concomitant with mitochondrial depletion in spinal cords at
 tributable to an impairment of SIRT1/PGC-1a pathway. Treating X-ALD mice w
 ith resveratrol\, a SIRT1 activator\, or overexpressing SIRT1 by transgene
 sis\, normalises mitochondria numbers and respiration\, preserves energeti
 c homeostasis including NADH and ATP levels and most importantly\, halts a
 xonal degeneration and related motor disability. These results link mitoch
 ondria malfunction to axonal damage\, and underscore the Sirt1/PGC-a axis 
 as a pivotal therapeutic target for neurodegenerative disease.Bio:\nCurren
 tly: ICREA Professor of Research\, Neurometabolic Diseases Laboratory\, In
 stitut de Investigacio Biomedica de Bellvitge (IDIBELL)\, Hospital Duran i
  Reynals\, L'Hospitalet de Llobregat\n1993 Medical Degree from the Autonom
 ous University of Barcelona\, Spain\n1998 PhD Thesis Degree the DKFZ\, Uni
 versity Ruprechts-Karls\, Heidelberg\, Germany\n1998-2002 Postdoctoral fel
 low at the IGMBC\, Human Molecular Genetics Department\, Strasbourg\, Fran
 ce\n2002 Consultant in Medical Genetics\, Université Louis Pasteur and H
 ôpitaux Universitaires de\, Strasbourg\, France\n2003-2004 Junior Group L
 eader at the IGBMC\, Strasbourg\, France\n2005 ICREA Reseach Professor and
  Group Leader at IDIBELL\, L'Hospitalet de Llobregat\, Barcelona\, SpainLi
 nk to Speaker's Lab websiteLink to PEROXISOME database (PeroxisomeDB)
LOCATION:AI 1153 https://plan.epfl.ch/?room==AI%201153
STATUS:CONFIRMED
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