Inborn Errors of Metabolism
Products for Inborn Errors of Metabolism
- Cat.No. Product Name Information
-
GC19941
DL-Leucic Acid
-
GC52404
γ-Glu-Phe (trifluoroacetate salt)
γ-Glutamylphenylalanine
A dipeptide with metabolism-altering activity
-
GC42051
1-β-D-Glucosylsphingosine (d18:1)
Glucosyl-C18-Sphingosine, Glucosylsphingosine (d18:1), 1-β-D-Glucosylsphingosine (synthetic)
1-β-D-Glucosylsphingosine is a glucosylsphingosine, which are deacetylated lysolipid derivatives of glucocerebrosides.
-
GC18609
1-Deoxysphinganine (m18:0)
1-deoxySA, ES-285, Spisulosine
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.
-
GC41907
13C C16 Sphingomyelin (d18:1/16:0)
Palmitoyl Sphingomyelin-13C, N-Palmitoyl-D-erythro-Sphingosylphosphorylcholine-13C, SM(d18:1/16:0-13C), Sphingomyelin (d18:1/16:0-13C)
13C C16 Sphingomyelin is an isotopically enriched form of C16 sphingomyelin with carbon-13 occurring on the fatty acid portion.
-
GC52122
2’-Deoxyadenosine-5’-diphosphate (sodium salt)
dADP, 2'-deoxy-ADP
A nucleotide diphosphate
-
GC45321
2',3',5'-Triacetyluridine
-
GC46540
2'-Deoxyadenosine-5'-triphosphate (sodium salt hydrate)
A purine nucleotide
-
GC49840
2-Methylbutyrylglycine
2-MBG, N-sec-Valerylglycine
A metabolite of isoleucine
-
GC52083
21-Deoxycortisone
21-Desoxycortisone, NSC 38722
A corticosteroid metabolite of 11-keto progesterone
-
GC41614
24-dehydro Cholesterol
Desmosterol
24-dehydro Cholesterol is a molecule similar to cholesterol.
-
GC46243
24-dehydro Cholesterol-d6
Desmosterol-d6
An internal standard for the quantification of 24-dehydro cholesterol
-
GC46068
3β-OH-7-Oxocholenic Acid
3β-hydroxy-7-oxo-5-Cholenoic Acid, 3β-hydroxy-7-Oxochol-5-enoic Acid
A bile acid
-
GC46584
3-Aminoisobutyric Acid (sodium salt)
β-Aminoisobutyric Acid, BAIBA
A neuropeptide with diverse biological activities
-
GC42292
3-Hydroxypropionic Acid (sodium salt)
Sodium β-hydroxypropionate
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.
-
GC49149
3-Methylglutaconic Acid
β-Methylglutaconic Acid, 3-MGA, 3-MGC Acid, NSC 249232
A branched-chain organic acid
-
GC41582
4(Z),7(Z)-Decadienoic Acid
4(Z),7(Z)-Decadienoic acid is a polyunsaturated fatty acid (PUFA).
-
GC42445
4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt)
4-MU β-D-galactopyranoside-6-sulfate, 4-Methylumbelliferyl Gal-6S, 4-MU-Gal-6S
4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt) is a fluorescent dye.
-
GC42442
4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (sodium salt)
4-MU-α-GlcNS
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.
-
GC40510
4-Methylumbelliferyl-α-D-Galactopyranoside
MU-α-Gal, 4-MU-α-Gal
4-Methylumbelliferyl-α-D-galactopyranoside (4-MU-α-Gal) is a fluorogenic substrate of α-galactosidase.
-
GC19852
4-Methylumbelliferyl-α-D-glucopyranoside
4-Methylumbelliferyl-α-D-Glucose, 4-Methylumbelliferyl-α-D-Glucoside, 4-MU-α-D-Glucopyranoside
-
GC11870
4-Methylumbelliferyl-β-D-Glucopyranoside
4-MU-GLU;4-MU-β-Gluc;4-MUG;MU-GLU
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.
-
GC49499
5(Z)-Dodecenoic Acid
C12:1 n-7, C12:1(5Z), cis-5-Dodecenoic Acid
5(Z)-Dodecenoic Acid is an endogenous metabolite with inhibitory activities against COX-I and COX-II.
-
GC46735
7-Biopterin
Primapterin, L-Primapterin
A pterin
-
GC46242
7-dehydro Cholesterol-d7
Provitamin D3-d7
7-dehydro Cholesterol (7-DHC) is an immediate precursor of cholesterol
-
GC42695
Acetoacetyl Coenzyme A (sodium salt hydrate)
Acetoacetyl-CoA
Acetoacetyl coenzyme A (acetoacetyl-CoA) is a precursor to HMG-CoA in the isoprenoid pathway.
-
GC46083
Adenylosuccinic Acid
Aspartyl Adenylate
Adenylosuccinic acid is a purine nucleotide and an intermediate in the purine nucleotide cycle.
-
GC42963
BODIPY-C12 Ceramide (d18:1/12:0)
BODIPY-C12 Ceramide, BODIPY-Ceramide (d18:1/12:0), BODIPY-Cer(d18:1/12:0), BODIPY-N-Lauroyl-D-erythro-Sphingosine, B12Cer
BODIPY-C12 Ceramide (d18:1/12:0) is a fluorescently labeled C12 ceramide.
-
GC43006
C12 Ceramide (d18:1/12:0)
Cer(d18:1/12:0), C12 Ceramide, Ceramide (d18:1/12:0), N-Lauroyl-D-erythro-Sphingosine
C12 Ceramide (d18:1/12:0) (N-Lauroyl-D-erythro-sphingosine), a naturally occurring ceramide, is formed by hydrolysis of C12 sphingomyelin.
-
GC43032
C16 Globotriaosylceramide (d18:1/16:0)
C16 Ceramide Trihexoside (d18:1/16:0), Gb3 (d18:1/16:0), N-Hexadecanoyl-ceramide trihexoside
C16 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from C16 lactosylceramide.
-
GC46980
C16 Globotriaosylceramide-d9 (d18:1/16:0-d9)
C16 Ceramide Trihexoside (d18:1/16:0-d9), Gb3 (d18:1/16:0-d9), N-Hexadecanoyl-d9 (13,13,14,14,15,15,16,16,16)-ceramide Trihexoside
A neuropeptide with diverse biological activities
-
GC43033
C16 Lactosylceramide (d18:1/16:0)
N-Hexadecanoyl Lactosylceramide, LacCer(d18:1/16:0), Lactosylceramide (d18:1/16:0), C16 Lactosyl(β) Ceramide (d18:1/16:0)
C16 Lactosylceramide is an endogenous bioactive sphingolipid.
-
GC46982
C16 Lactosylceramide-d3 (d18:1/16:0-d3)
C16 Lactosyl(β) Ceramide-d3 (d18:1/16:0-d3), LacCer(d18:1/16:0-d3), Lactosylceramide-d3 (d18:1/16:0-d3), N-ω-CD3-Hexadecanoyl-lactosylceramide, N-Hexadecanoyl Lactosylceramide-d3
A neuropeptide with diverse biological activities
-
GC43035
C16 Sphingomyelin (d18:1/16:0)
Palmitoyl Sphingomyelin, N-Palmitoyl-D-erythro-Sphingosylphosphorylcholine, SM(d18:1/16:0), Sphingomyelin (d18:1/16:0)
C16 Sphingomyelin (d18:1/16:0) is a form of sphingomyelin containing palmitate (16:0) at the variable acylation position.
-
GC45661
C16 Sphingomyelin-d9 (d18:1/16:0-d9)
Palmitoyl Sphingomyelin-d9
A neuropeptide with diverse biological activities
-
GC43038
C17 Ceramide (d18:1/17:0)
Cer(d18:1/17:0), Ceramide (d18:1/17:0), N-heptadecanoyl-D-erythro-Sphingosine
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)
C17 Ceramide Trihexoside (d18:1/17:0), N-Heptadecanoyl Ceramide Trihexoside
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.
-
GC46985
C18 3'-sulfo Galactosylceramide-d3 (d18:1/18:0-d3)
N-Octadecanoyl Sulfatide-d3, N-ω-CD3-Octadecanoyl-sulfatide
A neuropeptide with diverse biological activities
-
GC43049
C18 Globotriaosylceramide (d18:1/18:0)
C18 Ceramide Trihexoside (d18:1/18:0), Gb3(d18:1/18:0), N-Octadecanoyl-ceramide trihexoside
C18 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from lactosylceramide.
-
GC46995
C18 Globotriaosylceramide-d3 (d18:1/18:0-d3)
N-ω-CD3-Octadecanoyl-ceramide Trihexoside, Galα1-4Galβ1-4Glcβ-Cer(d18:1/18:0) d3, Gb3(d18:1/18:0-d3), Globotriaosylceramide-d3 (d18:1/18:0-d3)
A neuropeptide with diverse biological activities
-
GC43059
C2 Adamantanyl Galactosylceramide (d18:1/2:0)
C2 Adamantanyl Galactosylceramide (d18:1/2:0) (AdaGalCer) is a bioactive sphingolipid.
-
GC43068
C20 Sphingomyelin (d18:1/20:0)
N-eicosanoyl-D-erythro-Sphingosylphosphorylcholine, SM(d18:1/20:0)
C20 Sphingomyelin is a naturally occurring sphingolipid.
-
GC40041
Cholesteryl Lignocerate
24:0 CE, 24:0 Cholesterol ester
Cholesteryl lignocerate is a cholesterol ester that has been found in human meibum.
-
GC47115
Coenzyme B12 (hydrate)
Adenosylcobalamin, AdoCbl, Cobamamide, Dibenzcozamide, LM-176
A neuropeptide with diverse biological activities
-
GC47189
D-erythro/L-threo Lysosphingomyelin (d18:1)
D-erythro/L-threo Lyso SM(18:1), D-erythro/L-threo Sphingosine-1-Phosphocholine (d18:1), D-erythro/L-threo-Sphingosylphosphorylcholine
A mixture of bioactive sphingolipids
-
GC47204
D-Fructose-13C6
D-(-)-Fructose-13C6, D-(-)-Levulose-13C6
D-Fructose-13C6, a 13C-labeled D-Fructose, acts as an internal reference for fructose and xylitol quantitation.
-
GC49325
Dihydroxyacetone Phosphate (magnesium salt hydrate)
DHAP, Glycerone Phosphate
A glycerolipid precursor
-
GC43486
DL-Cystathionine (hydrochloride)
DL-Allocystathionine
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).
-
GC47286
Eliglustat-d15 (tartrate)
Genz 99067-d15 tartrate
An internal standard for the quantification of eliglustat
-
GC18469
Galactosylcerebrosides (bovine)
Galactosylcerebroside, Cerebroside, Ganglioside GL1b
Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with hydroxy and non-hydroxy fatty acids.
-
GC43721
Galactosylcerebrosides (hydroxy)
Cerebrosides; Phrenosin (bottom spot)
Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid.
-
GC43722
Galactosylcerebrosides (non-hydroxy)
Cerebrosides; Kerasin (top spot)
Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid.
-
GC43723
Galactosylsphingosine (d18:1)
Galactosylsphingosine, Psychosine
Galactosylsphingosine (d18:1) (Galactosylsphingosine), a substrate of the galactocerebrosidase (GALC) enzyme, is a potential biomarker for Krabbe disease.
-
GC43727
Ganglioside GD1a mixture (sodium salt)
Ganglioside B1
Ganglioside GD1a is a sialic acid-containing glycosphingolipid found in brain, erythrocytes, bone marrow, testis, spleen, and liver.
-
GC47392
Ganglioside GM1 Mixture (ovine) (ammonium salt)
GM1, Monosialoganglioside GM1
A mixture of ganglioside GM1
-
GC47393
Ganglioside GM1 Mixture (porcine brain) (ammonium salt)
GM1, Monosialoganglioside GM1
A mixture of ganglioside GM1
-
GC40016
Ganglioside GM2 Mixture (sodium salt)
GM2 Mixture, Monosialoganglioside GM2 Mixture
Ganglioside GM2 is a glycosphingolipid component of cellular membranes, primarily the plasma membrane.
-
GC52492
Globotriaosylceramide (hydroxy) (porcine RBC)
Ceramide Trihexosides (bottom spot) (porcine)
A sphingolipid
-
GC52491
Globotriaosylceramide (non-hydroxy) (porcine RBC)
Ceramide Trihexosides (top spot) (porcine)
A sphingolipid
-
GC43758
Globotriaosylceramides (hydroxy) (porcine)
Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides.
-
GC43760
Globotriaosylceramides (porcine)
Ceramide Trihexosides (porcine)
Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides.
-
GC43768
Glucocerebrosides (Gaucher's spleen)
Ganglioside GL1a, Gaucher cerebroside, GluCers (Gaucher's spleen), Glucocerebrosides, Glucosylceramides (Gaucher's spleen)
Glucocerebrosides are formed by the tethering of glucose to a ceramide by glucosylceramide synthase.
-
GC52493
Glucosylceramide (bovine buttermilk)
GluCers (buttermilk), Glucocerebrosides (buttermilk)
A sphingolipid
-
GC52219
Heptadecanoyl-L-carnitine (chloride)
CAR 17:0, C17:0 Carnitine, L-Carnitine heptadecanoyl ester, L-Heptadecanoylcarnitine
A long-chain acylcarnitine
-
GC52231
Heptadecanoyl-L-carnitine-d3 (chloride)
CAR 17:0-d3, C17:0 Carnitine-d3, L-Carnitine heptadecanoyl ester-d3, L-Heptadecanoylcarnitine-d3
An internal standard for the quantification of heptadecanoyl-L-carnitine
-
GC49479
Hypoxanthine-d4
6-Hydroxypurine-d4
An internal standard for the quantification of hypoxanthine
-
GC43914
Isobutyryl Coenzyme A (sodium salt)
Isobutyryl-CoA
Isobutyryl coenzyme A (isobutyryl-CoA) is a short-chain branched acyl CoA.
-
GC52009
Isovaleryl-DL-carnitine-d9 (chloride)
CAR 5:0-d9, C5:0 Carnitine-d9, DL-Carnitine isovaleryl ester-d9, DL-Isovalerylcarnitine-d9
An internal standard for the quantification of isovaleryl-DL-carnitine
-
GC52323
L-Isoleucine-13C6
L-(+)-Isoleucine-13C6
An internal standard for the quantification of L-isoleucine
-
GC49782
L-Phenylalanine-13C6
(-)-Phenylalanine-13C6
-
GC44101
Lyso-Globotriaosylceramide (d18:1)
Globotriaosylsphingosine (d18:1), Lyso-Ceramide Trihexoside, Lyso-Gb3
Lyso-globotriaosylceramide is a form of globotriaosylceramide that is lacking the fatty acyl group.
-
GC48345
Lyso-Monosialoganglioside GM2 (ammonium salt)
Lysoganglioside GM2, Lyso-GM2, lyso-Monosialoganglioside GM2
-
GC44105
Lysosphingomyelin (d18:1)
D-erythro Lyso SM(18:1), D-erythro-Sphingophosphorylcholine, D-erythro Sphingosine-1-Phosphocholine (d18:1), Lyso-SM(d18:1), Lysosphingomyelin (d18:1)
Sphingomyelins are complex membrane lipids composed of phosphorylcholine, sphingosine, and an acylated group, such as a fatty acid.
-
GC19926
Methyl malonic acid
Dimethyl Malonate; Malonic Acid Dimethyl Ester; Methyl Malonate
-
GC49472
Methylmalonic Acid-d3
Isosuccinic Acid-d3, Methylmalonate-d3, MMA-d3
Methylmalonic acid-d3 (Methylpropanedioic acid-d3) is the deuterium labeled Methylmalonic acid. Methylmalonic acid (Methylmalonate) is an indicator of Vitamin B-12 deficiency in cancer.
-
GC49246
N-(3-Phenylpropionyl)glycine
An acylglycine
-
GC49388
N-Butyldeoxynojirimycin-d9 (hydrochloride)
Miglustat-d9, NB-DNJ-d9
An internal standard for the quantification of N-butyldeoxynojirimycin
-
GC49240
N-Propionylglycine
NSC 158539
A glycine-conjugated form of propionic acid
-
GC47779
Nitisinone-13C6
NTBC-13C6
An internal standard for the quantification of nitisinone
-
GC49323
Norcholic Acid
23-Norcholic Acid
A bile acid and derivative of cholic acid
-
GC46187
Norhyodeoxycholic Acid
3α,6α-Dihydroxy-24-nor-5β-cholan-23-oic Acid, NHDCA
A synthetic bile acid
-
GC46192
Octanoic Acid-13C
Caprylic Acid-13C
An internal standard for the quantification of octanoic acid
-
GC45982
Octanoic Acid-d15
Caprylic Acid-d15
An internal standard for the quantification of octanoic acid
-
GC45996
Octanoic Acid-d2
Caprylic Acid-d2
An internal standard for the quantification of octanoic acid
-
GC45813
Octanoic Acid-d3
Caprylic Acid-d3
An internal standard for the quantification of octanoic acid
-
GC44488
Octanoyl Coenzyme A (sodium salt)
Octanoyl-CoA
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)
Phytanic Acid Coenzyme A Ester (triethylammonium salt), phytanoyl-CoA (triethylammonium salt)
Phytanic acid is a saturated 20-carbon branched-chain fatty acid that can only be derived from dietary sources.
-
GC48724
Pregnanetriol
NSC 52760, 5β-Pregnane-3α,17α,20α-triol
A metabolite of 17α-hydroxyprogesterone
-
GC45981
Pristanic Acid-d3
An internal standard for the quantification of pristanic acid
-
GC46215
S-Acetyl-L-glutathione
S-Acetylglutathione
A derivative of glutathione
-
GC52496
Sulfatide (bovine) (sodium salt)
Galactosylceramide I3-sulfate, Ganglioside SM4, I3SO3-GalCer, SM4, Sulfated Galactocerebroside, 3-O-Sulfogalactosylceramide
A mixture of isolated bovine sulfatides
-
GC40341
Tetrahydro-11-deoxy Cortisol
NSC 53901, 5β-Pregnane-3α,17α,21-triol-20-one, Tetrahydrodeoxycortisol, THS
Tetrahydro-11-deoxy cortisol (THS) is the primary urinary metabolite of 11-deoxycortisol.
-
GC91083
Tiopronin-Cysteine Disulfide
A metabolite of tiopronin
-
GC45945
Trihydroxycholestanoic Acid
Trihydroxycoprostanic Acid
A cholic acid intermediate
-
GC49328
UDP-α-D-Galactose (sodium salt)
UDP-Gal, Uridine-5'-diphosphate-α-D-Galactose
A nucleotide sugar
-
GC26265
α-Hydroxyglutaric acid disodium
2-Hydroxyglutarate disodium; 2-Hydroxyglutaric acid disodium; 2-Hydroxypentanedioic acid disodium
α-Hydroxyglutaric acid (2-Hydroxyglutarate) disodium is an α-hydroxy acid form of glutaric acid.