[图书][B] Psychosine-Based Lipid Raft Pathology: A Crossroads for Krabbe Disease

AB White - 2011 - search.proquest.com
Lipid rafts (LRs) are membrane realms characterized by high concentrations of cholesterol
and sphingolipids. Often, they are portrayed as scaffolds on which many different signaling …

Psychosine accumulates in membrane microdomains in the brain of krabbe patients, disrupting the raft architecture

AB White, MI Givogri, A Lopez-Rosas… - Journal of …, 2009 - Soc Neuroscience
Lipid rafts (LRs) are membrane realms characterized by high concentrations of cholesterol
and sphingolipids. Often, they are portrayed as scaffolds on which many different signaling …

Persistence of psychosine in brain lipid rafts is a limiting factor in the therapeutic recovery of a mouse model for Krabbe disease

AB White, F Galbiati, MI Givogri… - Journal of …, 2011 - Wiley Online Library
Sphingolipids are intrinsic components of membrane lipid rafts. The abnormal accumulation
of these molecules may introduce architectural and functional changes in these domains …

Extracellular Vesicles as Vehicles of Toxicity in Models of Krabbe Disease

C Reiter - 2022 - search.proquest.com
Krabbe's Disease is caused by mutations in the gene encoding the lysosomal enzyme
galactosylceramidase, leading to loss of normal catabolism of the prominent myelin lipid …

Psychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe's disease

L D'Auria, C Reiter, E Ward, AL Moyano, MS Marshall… - PLoS …, 2017 - journals.plos.org
In prior studies, our laboratory showed that psychosine accumulates and disrupts lipid rafts
in brain membranes of Krabbe's disease. A model of lipid raft disruption helped explaining …

[PDF][PDF] Krabbe disease: cytototicity and neuroinflammation

MV Gil Moreno de Mora de Haas - 2014 - ddd.uab.cat
Psychosine is a neurotoxic lipid, which accumulates in oligodendrocytes and Schwann cells.
The elevated levels of the cytotoxic lipid may cause the death in the myelin-forming cells …

The pathogenic sphingolipid psychosine is secreted in extracellular vesicles in the brain of a mouse model of Krabbe disease

CR Reiter, R Rebiai, A Kwak, J Marshall… - ASN …, 2022 - journals.sagepub.com
Psychosine exerts most of its toxic effects by altering membrane dynamics with increased
shedding of extracellular vesicles (EVs). In this study, we discovered that a fraction of …

Developmental and Pathological Effects of Psychosine on Oligodendrocytes of the Twitcher Mouse

H Zhu - 2013 - indigo.uic.edu
Psychosine is a lipid raft-associated cytotoxic sphingolipid that accumulates in individual
nervous system affected with Krabbe disease, a deficiency of lysosomal …

[PDF][PDF] Pathophysiology of Krabbe disease

A Davenport, P Williamson… - Orbit …, 2011 - openjournals.library.sydney.edu.au
Krabbe disease is a rapidly progressive lysosomal storage disorder that primarily affects
infants. Accumulation of psychosine, a cytotoxic substrate, in the myelin-forming cells results …

Role of endogenous psychosine accumulation in oligodendrocyte differentiation and survival: implication for Krabbe disease

JS Won, J Kim, MK Paintlia, I Singh, AK Singh - Brain research, 2013 - Elsevier
Krabbe disease is a lethal, demyelinating condition caused by genetic deficiency of
galactocerebrosidase (GALC) and resultant accumulation of its cytotoxic substrate …