Inborn Errors of Metabolism
Products Related to Inborn Errors of Metabolism(96)
- GC43038C17 Ceramide (d18:1/17:0)CAS: 67492-16-4Purity: >98.00%
C17 Ceramide (d18:1/17:0) is a synthetic ceramide containing a non-natural C17:0 fatty acid acylated to sphingosine.
- GC43041C17 Globotriaosylceramide (d18:1/17:0)CAS: 536745-81-0Purity: >98.00%
C17 Globotriaosylceramide is a sphingolipid that has been used as an internal standard for the quantification of globotriaosylceramides in plasma and urine from patients with Fabry disease by GC-MS.
- GC43049C18 Globotriaosylceramide (d18:1/18:0)CAS: 69283-33-6Purity: >98.00%
C18 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from lactosylceramide.
- GC43059C2 Adamantanyl Galactosylceramide (d18:1/2:0)CAS: 574738-16-2Purity: >98.00%
C2 Adamantanyl Galactosylceramide (d18:1/2:0) (AdaGalCer) is a bioactive sphingolipid.
- GC43068C20 Sphingomyelin (d18:1/20:0)CAS: 121999-68-6Purity: >98.00%
C20 Sphingomyelin is a naturally occurring sphingolipid.
- GC43486DL-Cystathionine (hydrochloride)Purity: >95.00%
DL-Cystathionine is an intermediate in the synthesis of cysteine that is produced from homocysteine and serine by the action of cystathionine-β-synthase (CBS).
- GC43721Galactosylcerebrosides (hydroxy)CAS: 37211-11-3Purity: >98.00%
Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid.
- GC43722Galactosylcerebrosides (non-hydroxy)CAS: 536-13-0Purity: >98.00%
Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid.
- GC43723Galactosylsphingosine (d18:1)CAS: 2238-90-6Purity: >95.00%
Galactosylsphingosine (d18:1) (Galactosylsphingosine), a substrate of the galactocerebrosidase (GALC) enzyme, is a potential biomarker for Krabbe disease.
- GC43727Ganglioside GD1a mixture (sodium salt)CAS: 12707-58-3Purity: >98.00%
Ganglioside GD1a is a sialic acid-containing glycosphingolipid found in brain, erythrocytes, bone marrow, testis, spleen, and liver.
- GC43758Globotriaosylceramides (hydroxy) (porcine)Purity: >98.00%
Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides.
- GC43760Globotriaosylceramides (porcine)CAS: 71965-57-6Purity: >98.00%
Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides.
- GC43768Glucocerebrosides (Gaucher's spleen)CAS: 85305-87-9Purity: >98.00%
Glucocerebrosides are formed by the tethering of glucose to a ceramide by glucosylceramide synthase.
- GC43914Isobutyryl Coenzyme A (sodium salt)Purity: >95.00%
Isobutyryl coenzyme A (isobutyryl-CoA) is a short-chain branched acyl CoA.
- GC44101Lyso-Globotriaosylceramide (d18:1)CAS: 126550-86-5Purity: >98.00%
Lyso-globotriaosylceramide is a form of globotriaosylceramide that is lacking the fatty acyl group.
- GC44105Lysosphingomyelin (d18:1)CAS: 1670-26-4Purity: >98.00%
Sphingomyelins are complex membrane lipids composed of phosphorylcholine, sphingosine, and an acylated group, such as a fatty acid.
- GC44488Octanoyl Coenzyme A (sodium salt)Purity: >95.00%
Octanoyl coenzyme A (octanoyl-CoA) is a medium-chain acyl CoA and a metabolic intermediate in mitochondrial fatty acid β-oxidation.
- GC44639phytanoyl-Coenzyme A (triethylammonium salt)Purity: >95.00%
Phytanic acid is a saturated 20-carbon branched-chain fatty acid that can only be derived from dietary sources.
- GC45661C16 Sphingomyelin-d9 (d18:1/16:0-d9)CAS: 2415226-88-7Purity: >99.00%
A neuropeptide with diverse biological activities
- GC45813Octanoic Acid-d3CAS: 156779-05-4Purity: >99.00%
An internal standard for the quantification of octanoic acid
- GC45981Pristanic Acid-d3CAS: 1383920-14-6Purity: >99.00%
An internal standard for the quantification of pristanic acid
- GC45982Octanoic Acid-d15CAS: 69974-55-6Purity: >99.00%
An internal standard for the quantification of octanoic acid
| Catalog No. | Product Name | CAS No. | Purity | Structure |
|---|---|---|---|---|
| GC43038 | C17 Ceramide (d18:1/17:0) | 67492-16-4 | >98.00% | |
C17 Ceramide (d18:1/17:0) is a synthetic ceramide containing a non-natural C17:0 fatty acid acylated to sphingosine. | ||||
| GC43041 | C17 Globotriaosylceramide (d18:1/17:0) | 536745-81-0 | >98.00% | |
C17 Globotriaosylceramide is a sphingolipid that has been used as an internal standard for the quantification of globotriaosylceramides in plasma and urine from patients with Fabry disease by GC-MS. | ||||
| GC43049 | C18 Globotriaosylceramide (d18:1/18:0) | 69283-33-6 | >98.00% | |
C18 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from lactosylceramide. | ||||
| GC43059 | C2 Adamantanyl Galactosylceramide (d18:1/2:0) | 574738-16-2 | >98.00% | |
C2 Adamantanyl Galactosylceramide (d18:1/2:0) (AdaGalCer) is a bioactive sphingolipid. | ||||
| GC43068 | C20 Sphingomyelin (d18:1/20:0) | 121999-68-6 | >98.00% | |
C20 Sphingomyelin is a naturally occurring sphingolipid. | ||||
| GC43486 | DL-Cystathionine (hydrochloride) | - | >95.00% | |
DL-Cystathionine is an intermediate in the synthesis of cysteine that is produced from homocysteine and serine by the action of cystathionine-β-synthase (CBS). | ||||
| GC43721 | Galactosylcerebrosides (hydroxy) | 37211-11-3 | >98.00% | |
Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid. | ||||
| GC43722 | Galactosylcerebrosides (non-hydroxy) | 536-13-0 | >98.00% | |
Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid. | ||||
| GC43723 | Galactosylsphingosine (d18:1) | 2238-90-6 | >95.00% | |
Galactosylsphingosine (d18:1) (Galactosylsphingosine), a substrate of the galactocerebrosidase (GALC) enzyme, is a potential biomarker for Krabbe disease. | ||||
| GC43727 | Ganglioside GD1a mixture (sodium salt) | 12707-58-3 | >98.00% | |
Ganglioside GD1a is a sialic acid-containing glycosphingolipid found in brain, erythrocytes, bone marrow, testis, spleen, and liver. | ||||
| GC43758 | Globotriaosylceramides (hydroxy) (porcine) | - | >98.00% | |
Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides. | ||||
| GC43760 | Globotriaosylceramides (porcine) | 71965-57-6 | >98.00% | |
Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides. | ||||
| GC43768 | Glucocerebrosides (Gaucher's spleen) | 85305-87-9 | >98.00% | |
Glucocerebrosides are formed by the tethering of glucose to a ceramide by glucosylceramide synthase. | ||||
| GC43914 | Isobutyryl Coenzyme A (sodium salt) | - | >95.00% | |
Isobutyryl coenzyme A (isobutyryl-CoA) is a short-chain branched acyl CoA. | ||||
| GC44101 | Lyso-Globotriaosylceramide (d18:1) | 126550-86-5 | >98.00% | |
Lyso-globotriaosylceramide is a form of globotriaosylceramide that is lacking the fatty acyl group. | ||||
| GC44105 | Lysosphingomyelin (d18:1) | 1670-26-4 | >98.00% | |
Sphingomyelins are complex membrane lipids composed of phosphorylcholine, sphingosine, and an acylated group, such as a fatty acid. | ||||
| GC44488 | Octanoyl Coenzyme A (sodium salt) | - | >95.00% | |
Octanoyl coenzyme A (octanoyl-CoA) is a medium-chain acyl CoA and a metabolic intermediate in mitochondrial fatty acid β-oxidation. | ||||
| GC44639 | phytanoyl-Coenzyme A (triethylammonium salt) | - | >95.00% | |
Phytanic acid is a saturated 20-carbon branched-chain fatty acid that can only be derived from dietary sources. | ||||
| GC45321 | 2',3',5'-Triacetyluridine | 4105-38-8 | >98.00% | |
| ||||
| GC45661 | C16 Sphingomyelin-d9 (d18:1/16:0-d9) | 2415226-88-7 | >99.00% | |
A neuropeptide with diverse biological activities | ||||
| GC45813 | Octanoic Acid-d3 | 156779-05-4 | >99.00% | |
An internal standard for the quantification of octanoic acid | ||||
| GC45945 | Trihydroxycholestanoic Acid | 547-98-8 | >95.00% | |
A cholic acid intermediate | ||||
| GC45981 | Pristanic Acid-d3 | 1383920-14-6 | >99.00% | |
An internal standard for the quantification of pristanic acid | ||||
| GC45982 | Octanoic Acid-d15 | 69974-55-6 | >99.00% | |
An internal standard for the quantification of octanoic acid | ||||