Inborn Errors of Metabolism

4-Methylumbelliferyl-β-D-Glucopyranoside is a β-D-glucoside that serves as a fluorescent substrate for β-glucosidase, ​thereby allowing for the detection and analysis of​ this enzyme's activity.

Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with hydroxy and non-hydroxy fatty acids.

1-Deoxysphinganine (m18:0) (ES-285) is an antiproliferative (antitumoral) compound of marine origin. 1-Deoxysphinganine (m18:0) inhibits the growth of the prostate PC-3 and LNCaP cells through intracellular ceramide accumulation and PKCζ activation.

α-Hydroxyglutaric acid (2-Hydroxyglutarate) disodium is an α-hydroxy acid form of glutaric acid.

Ganglioside GM2 is a glycosphingolipid component of cellular membranes, primarily the plasma membrane.

Cholesteryl lignocerate is a cholesterol ester that has been found in human meibum.

Tetrahydro-11-deoxy cortisol (THS) is the primary urinary metabolite of 11-deoxycortisol.

4-Methylumbelliferyl-α-D-galactopyranoside (4-MU-α-Gal) is a fluorogenic substrate of α-galactosidase.

4(Z),7(Z)-Decadienoic acid is a polyunsaturated fatty acid (PUFA).

24-dehydro Cholesterol is a molecule similar to cholesterol.

13C C16 Sphingomyelin is an isotopically enriched form of C16 sphingomyelin with carbon-13 occurring on the fatty acid portion.

1-β-D-Glucosylsphingosine is a glucosylsphingosine, which are deacetylated lysolipid derivatives of glucocerebrosides.

3-Hydroxypropionic acid is a building block in the synthesis of a variety of compounds with industrial applications, including acrylic acid, acrylamide, and 1,3-propanediol.

4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (sodium salt) is a fluorogenic substrate of heparin sulphamidase, is desulfurized into 4-MU-α-GlcNH2.

4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt) is a fluorescent dye.

Acetoacetyl coenzyme A (acetoacetyl-CoA) is a precursor to HMG-CoA in the isoprenoid pathway.

BODIPY-C12 Ceramide (d18:1/12:0) is a fluorescently labeled C12 ceramide.

C12 Ceramide (d18:1/12:0) (N-Lauroyl-D-erythro-sphingosine), a naturally occurring ceramide, is formed by hydrolysis of C12 sphingomyelin.

C16 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from C16 lactosylceramide.

C16 Lactosylceramide is an endogenous bioactive sphingolipid.

C16 Sphingomyelin (d18:1/16:0) is a form of sphingomyelin containing palmitate (16:0) at the variable acylation position.