Psychosine | CAS#2238-90-6 | Endogenous cytotoxin

MedKoo Cat#: 585053 | Name: Psychosine

Featured

Description:

WARNING: This product is for research use only, not for human or veterinary use.

Psychosine is 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. Psychosine causes myelinating cells death and demyelination which recruits microglia/macrophages that fail to digest myelin debris and become globoid cells.

Chemical Structure

Psychosine

CAS#2238-90-6

Theoretical Analysis

MedKoo Cat#: 585053

Name: Psychosine

CAS#: 2238-90-6

Chemical Formula: C24H47NO7

Exact Mass: 461.3353

Molecular Weight: 461.64

Elemental Analysis: C, 62.44; H, 10.26; N, 3.03; O, 24.26

Price and Availability

Size	Price	Availability	Quantity
1mg	USD 230.00
5mg	USD 485.00
10mg	USD 745.00

Bulk Inquiry

Buy Now

Add to Cart

Related CAS #

No Data

Synonym

Psychosine; Galactosylsphingosine; Sphingosine galactoside

IUPAC/Chemical Name

beta-D-Galactopyranoside, 2-amino-3-hydroxy-4-octadecenyl, (R-(R*,S*-(E)))-

InChi Key

HHJTWTPUPVQKNA-PIIMIWFASA-N

InChi Code

InChI=1S/C24H47NO7/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-19(27)18(25)17-31-24-23(30)22(29)21(28)20(16-26)32-24/h14-15,18-24,26-30H,2-13,16-17,25H2,1H3/b15-14+/t18-,19+,20+,21-,22-,23+,24+/m0/s1

SMILES Code

O[C@H]([C@H]([C@H]([C@@H](CO)O1)O)O)[C@@H]1OC[C@H](N)[C@H](O)/C=C/CCCCCCCCCCCCC

Appearance

Solid powder

Purity

>98% (or refer to the Certificate of Analysis)

Shipping Condition

Shipped under ambient temperature as non-hazardous chemical. This product is stable enough for a few weeks during ordinary shipping and time spent in Customs.

Storage Condition

Dry, dark and at 0 - 4 C for short term (days to weeks) or -20 C for long term (months to years).

Solubility

To be determined

Shelf Life

>3 years if stored properly

Drug Formulation

This drug may be formulated in DMSO

Stock Solution Storage

0 - 4 C for short term (days to weeks), or -20 C for long term (months).

HS Tariff Code

2934.99.9001

More Info

Product Data

Product Data

Instruction

View handling instruction

Biological target:

Psychosine induces inflammation, cell death, and demyelination in mouse astrocytes and cerebellar slices. It inhibits PKC translocation, induces apoptosis in oligodendrocytes, and disrupts gene transcription. Elevated levels are linked to Krabbe disease, a lysosomal storage disorder.

In vitro activity:

This study investigated how psychosine contributes to Globoid Cell Leukodystrophy (GLD). Both psychosine and the enantiomer of psychosine disrupted membrane integrity, localized to lipid rafts, and hindered protein Kinase C translocation to the plasma membrane. This suggests that psychosine primarily exerts its toxicity through interactions with cell membranes. Reference: J Lipid Res. 2013 Dec;54(12):3303-11. https://pubmed.ncbi.nlm.nih.gov/24006512/

In vivo activity:

This study supports the use of dried blood spot (DBS) psychosine concentration as a secondary screening for newborns. It effectively identifies infants at high risk of infantile Krabbe disease, prompting urgent evaluation for hematopoietic stem cell transplantation. Elevated DBS psychosine concentration during the newborn period was found to be a highly specific marker for infantile Krabbe disease. Reference: Mol Genet Metab. 2017 Jul;121(3):271-278. https://pubmed.ncbi.nlm.nih.gov/28579020/

Preparing Stock Solutions

The following data is based on the product molecular weight 461.64 Batch specific molecular weights may vary from batch to batch due to the degree of hydration, which will affect the solvent volumes required to prepare stock solutions.

Concentration / Solvent Volume / Mass	1 mg	5 mg	10 mg
1 mM	1.15 mL	5.76 mL	11.51 mL
5 mM	0.23 mL	1.15 mL	2.3 mL
10 mM	0.12 mL	0.58 mL	1.15 mL
50 mM	0.02 mL	0.12 mL	0.23 mL

Formulation protocol:

1. Del Grosso A, Antonini S, Angella L, Tonazzini I, Signore G, Cecchini M. Lithium improves cell viability in psychosine-treated MO3.13 human oligodendrocyte cell line via autophagy activation. J Neurosci Res. 2016 Nov;94(11):1246-60. doi: 10.1002/jnr.23910. PMID: 27638607. 2. Hawkins-Salsbury JA, Parameswar AR, Jiang X, Schlesinger PH, Bongarzone E, Ory DS, Demchenko AV, Sands MS. Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture. J Lipid Res. 2013 Dec;54(12):3303-11. doi: 10.1194/jlr.M039610. Epub 2013 Sep 4. PMID: 24006512; PMCID: PMC3826678. 3. Guenzel AJ, Turgeon CT, Nickander KK, White AL, Peck DS, Pino GB, Studinski AL, Prasad VK, Kurtzberg J, Escolar ML, Lasio MLD, Pellegrino JE, Sakonju A, Hickey RE, Shallow NM, Ream MA, Orsini JJ, Gelb MH, Raymond K, Gavrilov DK, Oglesbee D, Rinaldo P, Tortorelli S, Matern D. The critical role of psychosine in screening, diagnosis, and monitoring of Krabbe disease. Genet Med. 2020 Jun;22(6):1108-1118. doi: 10.1038/s41436-020-0764-y. Epub 2020 Feb 24. PMID: 32089546. 4. Escolar ML, Kiely BT, Shawgo E, Hong X, Gelb MH, Orsini JJ, Matern D, Poe MD. Psychosine, a marker of Krabbe phenotype and treatment effect. Mol Genet Metab. 2017 Jul;121(3):271-278. doi: 10.1016/j.ymgme.2017.05.015. Epub 2017 May 22. PMID: 28579020; PMCID: PMC5548593.

In vitro protocol:

In vivo protocol:

1. Guenzel AJ, Turgeon CT, Nickander KK, White AL, Peck DS, Pino GB, Studinski AL, Prasad VK, Kurtzberg J, Escolar ML, Lasio MLD, Pellegrino JE, Sakonju A, Hickey RE, Shallow NM, Ream MA, Orsini JJ, Gelb MH, Raymond K, Gavrilov DK, Oglesbee D, Rinaldo P, Tortorelli S, Matern D. The critical role of psychosine in screening, diagnosis, and monitoring of Krabbe disease. Genet Med. 2020 Jun;22(6):1108-1118. doi: 10.1038/s41436-020-0764-y. Epub 2020 Feb 24. PMID: 32089546. 2. Escolar ML, Kiely BT, Shawgo E, Hong X, Gelb MH, Orsini JJ, Matern D, Poe MD. Psychosine, a marker of Krabbe phenotype and treatment effect. Mol Genet Metab. 2017 Jul;121(3):271-278. doi: 10.1016/j.ymgme.2017.05.015. Epub 2017 May 22. PMID: 28579020; PMCID: PMC5548593.

1: Evans LMP, Gawron J, Sim FJ, Feltri ML, Marziali LN. Human iPSC-derived myelinating organoids and globoid cells to study Krabbe Disease. bioRxiv [Preprint]. 2024 Jul 23:2024.07.19.604372. doi: 10.1101/2024.07.19.604372. PMID: 39091729; PMCID: PMC11291050. 2: Dubiela P, Szymanska-Rozek P, Hasinski P, Lipinski P, Kleinotiene G, Giersz D, Tylki-Szymanska A. Long- and Short-Term Glucosphingosine (lyso-Gb1) Dynamics in Gaucher Patients Undergoing Enzyme Replacement Therapy. Biomolecules. 2024 Jul 12;14(7):842. doi: 10.3390/biom14070842. PMID: 39062556; PMCID: PMC11275231. 3: Saldivia N, Heller G, Zelada D, Whitehair J, Venkat N, Konjeti A, Savitzky R, Samano S, Simchuk D, van Breemen R, Givogri MI, Bongarzone ER. Deficiency of galactosyl-ceramidase in adult oligodendrocytes worsens disease severity during chronic experimental allergic encephalomyelitis. Mol Ther. 2024 Jun 26:S1525-0016(24)00425-8. doi: 10.1016/j.ymthe.2024.06.035. Epub ahead of print. PMID: 38937968. 4: Marano M, Zizzo C, Malaguti MC, Bacchin R, Cavallieri F, De Micco R, Spagnolo F, Bentivoglio AR, Schirinzi T, Bovenzi R, Ramat S, Erro R, Sorrentino C, Sucapane P, Pilotto A, Lupini A, Magliozzi A, Di Vico I, Carecchio M, Bonato G, Cilia R, Colucci F, Tamma F, Caputo E, Mostile G, Arabia G, Modugno N, Zibetti M, Ceravolo MG, Tambasco N, Cossu G, Valzania F, Manganotti P, Di Lazzaro V, Zappia M, Fabbrini G, Tinazzi M, Tessitore A, Duro G, Di Fonzo A. Increased glucosylsphingosine levels and Gaucher disease in GBA1-associated Parkinson's disease. Parkinsonism Relat Disord. 2024 Jul;124:107023. doi: 10.1016/j.parkreldis.2024.107023. Epub 2024 Jun 1. PMID: 38843618. 5: Inamura N, Kawai T, Watanabe T, Aoki H, Aoyama M, Nakayama A, Matsuda J, Enokido Y. Promyelinating drugs ameliorate oligodendrocyte pathologies in a mouse model of Krabbe disease. Mol Genet Metab. 2024 Jul;142(3):108497. doi: 10.1016/j.ymgme.2024.108497. Epub 2024 May 15. PMID: 38763041. 6: Favret J, Nawaz MH, Patel M, Khaledi H, Gelb M, Shin D. Perinatal loss of galactosylceramidase in both oligodendrocytes and microglia is crucial for the pathogenesis of Krabbe disease in mice. Mol Ther. 2024 Jul 3;32(7):2207-2222. doi: 10.1016/j.ymthe.2024.05.019. Epub 2024 May 11. PMID: 38734898; PMCID: PMC11286809. 7: Guerra J, Belleri M, Paiardi G, Tobia C, Capoferri D, Corli M, Scalvini E, Ghirimoldi M, Manfredi M, Wade RC, Presta M, Mignani L. Impact of an irreversible β-galactosylceramidase inhibitor on the lipid profile of zebrafish embryos. Comput Struct Biotechnol J. 2024 Mar 30;23:1397-1407. doi: 10.1016/j.csbj.2024.03.023. PMID: 38596316; PMCID: PMC11002810. 8: Papakyriakopoulou P, Valsami G, Dev KK. The Effect of Donepezil Hydrochloride in the Twitcher Mouse Model of Krabbe Disease. Mol Neurobiol. 2024 Apr 1. doi: 10.1007/s12035-024-04137-0. Epub ahead of print. PMID: 38558359. 9: Béchet S, Dev KK. The Effects of the S1P Receptor Agonist Fingolimod (FTY720) on Central and Peripheral Myelin in Twitcher Mice. Biomedicines. 2024 Mar 6;12(3):594. doi: 10.3390/biomedicines12030594. PMID: 38540207; PMCID: PMC10967782. 10: Vernet Machado Bressan Wilke M, Iop GD, Faqueti L, Lemos da Silva LA, Kubaski F, Poswar FO, Michelin-Tirelli K, Randon D, Borelli WV, Giugliani R, Schwartz IVD. A Brazilian Rare-Disease Center's Experience with Glucosylsphingosine (lyso-Gb1) in Patients with Gaucher Disease: Exploring a Novel Correlation with IgG Levels in Plasma and a Biomarker Measurement in CSF. Int J Mol Sci. 2024 Mar 1;25(5):2870. doi: 10.3390/ijms25052870. PMID: 38474117; PMCID: PMC10931658. 11: Ketata I, Ellouz E. From pathological mechanisms in Krabbe disease to cutting-edge therapy: A comprehensive review. Neuropathology. 2024 Aug;44(4):255-277. doi: 10.1111/neup.12967. Epub 2024 Mar 6. PMID: 38444347. 12: Ray SK, Dasgupta S. Chromatographic Separation and Quantitation of Sphingolipids from the Central Nervous System or Any Other Biological Tissue. Methods Mol Biol. 2024;2761:149-157. doi: 10.1007/978-1-0716-3662-6_12. PMID: 38427236. 13: Del Grosso A, Carpi S, De Sarlo M, Scaccini L, Colagiorgio L, Alabed HBR, Angella L, Pellegrino RM, Tonazzini I, Emiliani C, Cecchini M. Chronic Rapamycin administration via drinking water mitigates the pathological phenotype in a Krabbe disease mouse model through autophagy activation. Biomed Pharmacother. 2024 Apr;173:116351. doi: 10.1016/j.biopha.2024.116351. Epub 2024 Feb 28. PMID: 38422660. 14: Matern D, Basheeruddin K, Klug TL, McKee G, Edge PU, Hall PL, Kurtzberg J, Orsini JJ. Newborn Screening for Krabbe Disease: Status Quo and Recommendations for Improvements. Int J Neonatal Screen. 2024 Jan 28;10(1):10. doi: 10.3390/ijns10010010. PMID: 38390974; PMCID: PMC10885092. 15: Furderer ML, Berhe B, Chen TC, Wincovitch S, Jiang X, Tayebi N, Sidransky E, Han TU. A Comparative Biochemical and Pathological Evaluation of Brain Samples from Knock-In Murine Models of Gaucher Disease. Int J Mol Sci. 2024 Feb 2;25(3):1827. doi: 10.3390/ijms25031827. PMID: 38339105; PMCID: PMC10855869. 16: Farah MH, Dali CÍ, Groeschel S, Moldovan M, Whiteman DAH, Malanga CJ, Krägeloh-Mann I, Li J, Barton N, Krarup C. Effects of sulfatide on peripheral nerves in metachromatic leukodystrophy. Ann Clin Transl Neurol. 2024 Feb;11(2):328-341. doi: 10.1002/acn3.51954. Epub 2023 Dec 26. PMID: 38146590; PMCID: PMC10863914. 17: Cabasso O, Kuppuramalingam A, Lelieveld L, Van der Lienden M, Boot R, Aerts JM, Horowitz M. Animal Models for the Study of Gaucher Disease. Int J Mol Sci. 2023 Nov 7;24(22):16035. doi: 10.3390/ijms242216035. PMID: 38003227; PMCID: PMC10671165. 18: Rasmussen CA, Quadri A, Vucko E, Kim K, Hickey R, Baker JJ, Charrow J, Prada CE. Treatment-naive and post-treatment glucosylsphingosine (lyso-GL1) levels in a cohort of pediatric patients with Gaucher disease. Mol Genet Metab. 2024 Jan;141(1):107736. doi: 10.1016/j.ymgme.2023.107736. Epub 2023 Nov 14. PMID: 38000346. 19: Bradbury AM, Bagel J, Swain G, Miyadera K, Pesayco JP, Assenmacher CA, Brisson B, Hendricks I, Wang XH, Herbst Z, Pyne N, Odonnell P, Shelton GD, Gelb M, Hackett N, Szabolcs P, Vite CH, Escolar M. Combination HSCT and intravenous AAV-mediated gene therapy in a canine model proves pivotal for translation of Krabbe disease therapy. Mol Ther. 2024 Jan 3;32(1):44-58. doi: 10.1016/j.ymthe.2023.11.014. Epub 2023 Nov 11. PMID: 37952085; PMCID: PMC10787152. 20: Maghazachi AA. Globoid Cell Leukodystrophy (Krabbe Disease): An Update. Immunotargets Ther. 2023 Oct 31;12:105-111. doi: 10.2147/ITT.S424622. PMID: 37928748; PMCID: PMC10625317.