INFIQC   05475
INSTITUTO DE INVESTIGACIONES EN FISICO- QUIMICA DE CORDOBA
Unidad Ejecutora - UE
congresos y reuniones científicas
Título:
EFFECT OF PSYCHOSINE ON MODEL LIPID MEMBRANES
Autor/es:
YENISLEIDY DE LAS MERCEDES ZULUETA DIAZ; MARÍA L. FANANI; SOFIA CABY
Lugar:
Buenos Aires
Reunión:
Congreso; Reunión Conjunta de Sociedades de BioCiencias; 2017
Resumen:
Psychosine o â-galactosylsphingosine (PSY) is an intermediate in the biosynthesis of sphingolipids occurring in the lysosome lumen. It is degraded by the enzyme â-galactosylceramidase. A deficient of this enzymatic activity results in the progressive accumulation of PSY 10- 100 folds above it normal concetration, being responsible for the Krabbe disease or Globoid cell leukodystrophy.This is an inherited autosomal disorder that leads to demyelination, infiltration of macro phages into the brain parenchyma and early death. Several evidences proposes that this amphiphilic molecule exerts its pathological effect by partitioning into membranes and affecting it function. Additionally, it is known that PSY is hemolytic and induces permeabilization of liposomes.