Psychosine is a highly cytotoxic lipid that accumulates in the nervous system in the absence of galactosylceramidase. [1] Chemically, it is a galactoside of sphingosine.

Psychosine is a glycosylsphingoid consisting of sphingosine having a beta-D-galactosyl residue attached at the 1-position. It has a role as a human metabolite. It is functionally related to a sphing-4-enine and a sphingosine. It is a conjugate base of a psychosine(1+).

Psychosine is 1 of 4 substrates degraded by GALC and is a neurotoxin at elevated concentrations. It has been shown to be elevated in patients with active Krabbe disease or with saposin A cofactor deficiency and, therefore, may be a useful biomarker for the presence of disease or disease progression.

Psychosine is a sphingolipid that targets the activation of pro-apoptotic pathways in oligodendrocytes and Schwann cells, leading to cell death and myelin loss. It interferes with mitochondrial potential, activates caspases, and causes membrane damage.

In the absence of GALC, the cytotoxic glycosphingolipid, psychosine (psy), accumulates in the nervous system. Psychosine accumulation preferentially affects oligodendrocytes, leading to progressive demyelination and infiltration of activated monocytes/macrophages into the CNS.

2020年2月24日 · Psychosine (PSY) was proposed as a disease marker useful to reduce the false positive rate for NBS and for disease monitoring. We report a highly sensitive PSY assay that allows identification of...

In this study, we provide, for the first time, longitudinal data on dried blood spot (DBS) psychosine concentrations in different Krabbe disease phenotypes for both untreated patients and those treated with hematopoietic stem cell transplantation (HSCT).

Krabbe disease happens when children are missing all or some of the GALC enzyme. GALC breaks down psychosine. When psychosine is not broken down, it builds up in the body and can lead to damage to the nerves. What health problems can …

2001年4月4日 · Psychosine is a lysolipid, with detergent-like properties, so its target might in principle be some aspect of membrane biophysics rather than a specific protein. While hunting for the ligands of an orphan receptor, Im et al. 2001 appear …

2018年2月26日 · This study measured the content of psychosine in multiple brain regions of the adult brain, finding that psychosine follows a robust pattern of distribution, enriched within the white matter of the cortex and the substantia nigra in …