Psychosine (Galactosylsphingosine)

An intermediate in the biosynthesis of cerebrosides. It is formed by reaction of sphingosine with UDP-galactose and then itself reacts with fatty acid-Coenzyme A to form the cerebroside.

Also Known As:

Networked: 188 relevant articles (9 outcomes, 19 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Bongarzone, Ernesto R: 21 articles (01/2022 - 01/2011)
2.	Sands, Mark S: 12 articles (01/2022 - 06/2010)
3.	Jiang, Xuntian: 9 articles (01/2022 - 01/2009)
4.	Singh, Inderjit: 9 articles (01/2015 - 05/2002)
5.	Givogri, Maria I: 8 articles (01/2022 - 01/2011)
6.	Nguyen, Duc: 7 articles (01/2022 - 01/2017)
7.	van Breemen, Richard: 7 articles (01/2022 - 07/2011)
8.	Lopez-Rosas, Aurora: 7 articles (01/2015 - 01/2011)
9.	Singh, Avtar K: 7 articles (01/2015 - 05/2002)
10.	Wrabetz, Lawrence: 6 articles (01/2022 - 11/2016)

Related Diseases

1.	Globoid Cell Leukodystrophy (Krabbe Disease) 10/01/2019 - "In total, these data clarify our understanding of psychosine synthesis, confirm the long-held psychosine hypothesis, and provide the impetus to discover safe and effective inhibitors of ACDase to treat Krabbe disease." 01/01/2022 - "In this study, we discovered that a fraction of psychosine produced in the brain of the Twitcher mouse, a model for Krabbe disease, is associated with secreted EVs. " 01/01/2017 - "Future studies should aim to better delineate the relationship between DBS psychosine concentration and disease onset in patients with later-onset forms of Krabbe disease." 01/01/1997 - "Previous studies have suggested that psychosine is the causative agent for the pathogenesis of Krabbe's disease. " 03/22/2023 - "These events were not dependent on the accumulation of psychosine, which was previously identified as a possible player in autophagic impairment in Krabbe disease. "
2.	Demyelinating Diseases (Demyelinating Disease) 11/15/2016 - "Accumulation of psychosine to the cytotoxic levels in KD patients is thought to cause dysfunctions in myelinating glial cells based on a comprehensive study of demyelination in KD. " 01/01/2023 - "The demyelination observed in Twi/Sap-D KO mice may be mediated by a psychosine-independent, Sap-D-dependent mechanism. " 01/01/2022 - "In contrast, GALC G41S/G41S mice have normal lifespan, modest decreases of GALC, and minimal psychosine accumulation, but develop adult mild inflammatory demyelination and slight declines in coordination, motor skills, and memory. " 10/01/2021 - "Moreover, we studied the effects of nanoparticles ex vivo using mouse cerebellar organotypic cultures, observing that nanoparticles prevented the demyelination and axonal damage caused by psychosine, as well as a moderate prevention of the astrocytic death. " 01/01/2021 - "Psychosine accumulation affects many different cellular pathways, leading to severe demyelination. "
3.	Neuroinflammatory Diseases 09/01/2012 - "Twitcher mice receiving both ERT and BMT had significantly increased lifespan, improved motor function, reduced psychosine levels, and reduced neuroinflammation in certain areas of the brain compared to untreated or ERT-treated Twitcher mice. " 04/22/2015 - "Simultaneously treating multiple pathogenic targets resulted in an unprecedented increase in life span with improved motor function, persistent GALC expression, nearly normal psychosine levels, and decreased neuroinflammation. " 09/01/2012 - "At 36 days of age, ERT-treated Twitcher mice had reduced psychosine levels, reduced neuroinflammation, improved motor function, and increased lifespan. " 01/01/2022 - "It prevented psychosine accumulation in the CSF, preserved peripheral nerve myelination, ambulation, and decreased brain neuroinflammation and demyelination, although some regions remained abnormal. " 10/01/2021 - "Hybrid Nanoparticles as a Novel Tool for Regulating Psychosine-Induced Neuroinflammation and Demyelination In Vitro and Ex vivo."
4.	Inflammation (Inflammations) 06/01/2008 - "This study delineates an explicit role for AMPK in psychosine induced inflammation in astrocytes without directly affecting the cell death of oligodendrocytes. " 07/06/2011 - "Although AAV2/5 alone provided supraphysiological levels of GALC activity and reduced psychosine levels in both the brain and spinal cord, it significantly increased CNS inflammation. " 11/01/2016 - "Inflammation in Krabbe's disease has been considered a secondary effect, resulting from cell-autonomous oligodendroglial cell death or myelin loss resulting from psychosine accumulation. " 01/01/2020 - "Herein, we demonstrate in the naturally occurring canine model of GLD that presymptomatic monotherapy with intrathecal AAV9 encoding canine GALC administered into the cisterna magna increased GALC enzyme activity, normalized psychosine concentration, improved myelination, and attenuated inflammation in both the CNS and PNS. " 12/01/2011 - "One of a family of devastating lysosomal storage disorders, Krabbe disease is characterized by demyelination, psychosine accumulation, and inflammation. "
5.	Lysosomal Storage Diseases (Lysosomal Storage Disease) 11/01/2016 - "Because substrate inhibition therapy has been shown to be effective in some human lysosomal storage diseases, we hypothesize that a substrate inhibition therapeutic approach might be appropriate to allow correction of the imbalance between formation and breakdown of glycosphingolipids and to prevent pathological storage of psychosine. " 11/01/2016 - "Globoid cell leukodystrophy (GLD) is a lysosomal storage disease caused by β-galactosylceramidase (GALC) deficiency and characterized by the accumulation of the neurotoxic metabolite psychosine in the central nervous system and peripheral tissues. " 01/01/2023 - "We have shown in vivo and in vitro previously that psychosine causes dysfunction of autophagy and the ubiquitin-proteasome system underlying the pathogenesis of globoid cell leukodystrophy (GLD), a devastating lysosomal storage disease complicated by global demyelination. " 01/01/2022 - "Krabbe disease is a lysosomal storage disease caused by mutations in the gene that encodes galactosylceramidase, in which galactosylsphingosine (psychosine) accumulation drives demyelination in the central and peripheral nervous systems, ultimately progressing to death in early childhood. " 11/01/2016 - "Krabbe's disease, also known as globoid cell leukodystrophy (GLD), is a lysosomal storage disease caused by the deficiency of the lysosomal enzyme β-galactocerebrosidase (GALC), resulting in severe neurological manifestations related to demyelination secondary to elevated galactosylsphingosine (psychosine) with its subsequent cytotoxicity. "

Related Drugs and Biologics

1.	Enzymes
2.	Lipids
3.	Glycosphingolipids
4.	Secretory Phospholipases A2
5.	Sphingolipids
6.	Phorbol Esters
7.	Free Radicals
8.	Dimethyl Sulfoxide (DMSO)
9.	Antioxidants
10.	Psychosine (Galactosylsphingosine)

Related Therapies and Procedures

1.	Therapeutics
2.	Bone Marrow Transplantation (Transplantation, Bone Marrow)
3.	Hematopoietic Stem Cell Transplantation
4.	Enzyme Replacement Therapy
5.	Cell Transplantation