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Home >> Signaling Pathways >> Metabolism >> Inborn Errors of Metabolism

Inborn Errors of Metabolism

Products for  Inborn Errors of Metabolism

  1. Cat.No. Product Name Information
  2. GC52404 γ-Glu-Phe (trifluoroacetate salt) A dipeptide with metabolism-altering activity γ-Glu-Phe (trifluoroacetate salt) Chemical Structure
  3. GC42051 1-β-D-Glucosylsphingosine (d18:1) 1-β-D-Glucosylsphingosine is a glucosylsphingosine, which are deacetylated lysolipid derivatives of glucocerebrosides. 1-β-D-Glucosylsphingosine (d18:1) Chemical Structure
  4. GC18609 1-Deoxysphinganine (m18:0) 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. 1-Deoxysphinganine (m18:0) Chemical Structure
  5. GC41907 13C C16 Sphingomyelin (d18:1/16:0) 13C C16 Sphingomyelin is an isotopically enriched form of C16 sphingomyelin with carbon-13 occurring on the fatty acid portion. 13C C16 Sphingomyelin (d18:1/16:0) Chemical Structure
  6. GC52122 2’-Deoxyadenosine-5’-diphosphate (sodium salt) A nucleotide diphosphate 2’-Deoxyadenosine-5’-diphosphate (sodium salt) Chemical Structure
  7. GC46540 2'-Deoxyadenosine-5'-triphosphate (sodium salt hydrate) A purine nucleotide 2'-Deoxyadenosine-5'-triphosphate (sodium salt hydrate) Chemical Structure
  8. GC49840 2-Methylbutyrylglycine A metabolite of isoleucine 2-Methylbutyrylglycine Chemical Structure
  9. GC52083 21-Deoxycortisone A corticosteroid metabolite of 11-keto progesterone 21-Deoxycortisone Chemical Structure
  10. GC41614 24-dehydro Cholesterol 24-dehydro Cholesterol is a molecule similar to cholesterol. 24-dehydro Cholesterol Chemical Structure
  11. GC46243 24-dehydro Cholesterol-d6 An internal standard for the quantification of 24-dehydro cholesterol 24-dehydro Cholesterol-d6 Chemical Structure
  12. GC46068 3β-OH-7-Oxocholenic Acid A bile acid 3β-OH-7-Oxocholenic Acid Chemical Structure
  13. GC42292 3-Hydroxypropionic Acid (sodium salt) 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. 3-Hydroxypropionic Acid (sodium salt) Chemical Structure
  14. GC49149 3-Methylglutaconic Acid A branched-chain organic acid 3-Methylglutaconic Acid Chemical Structure
  15. GC41582 4(Z),7(Z)-Decadienoic Acid 4(Z),7(Z)-Decadienoic acid is a polyunsaturated fatty acid (PUFA). 4(Z),7(Z)-Decadienoic Acid Chemical Structure
  16. GC42445 4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt) 4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt) is a fluorescent dye. 4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt) Chemical Structure
  17. GC42442 4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (sodium salt) 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 2-sulfamino-2-deoxy-α-D-Glucopyranoside (sodium salt) Chemical Structure
  18. GC40510 4-Methylumbelliferyl-α-D-Galactopyranoside 4-Methylumbelliferyl-α-D-galactopyranoside (4-MU-α-Gal) is a fluorogenic substrate of α-galactosidase. 4-Methylumbelliferyl-α-D-Galactopyranoside Chemical Structure
  19. GC11870 4-Methylumbelliferyl-β-D-Glucopyranoside

    4-Methylumbelliferyl-β-D-Glucopyranoside (4-MUG) is a fluorogenic substrate of β-glucosidase and β-glucocerebrosidase (also known as glucosylceramidase).

     4-Methylumbelliferyl-β-D-Glucopyranoside Chemical Structure
  20. GC49499 5(Z)-Dodecenoic Acid 5(Z)-Dodecenoic Acid is an endogenous metabolite with inhibitory activities against COX-I and COX-II. 5(Z)-Dodecenoic Acid Chemical Structure
  21. GC46735 7-Biopterin A pterin 7-Biopterin Chemical Structure
  22. GC46242 7-dehydro Cholesterol-d7

    7-dehydro Cholesterol (7-DHC) is an immediate precursor of cholesterol

     7-dehydro Cholesterol-d7 Chemical Structure
  23. GC42695 Acetoacetyl Coenzyme A (sodium salt hydrate)

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

     Acetoacetyl Coenzyme A (sodium salt hydrate) Chemical Structure
  24. GC42963 BODIPY-C12 Ceramide (d18:1/12:0) BODIPY-C12 Ceramide (B12Cer) is a fluorescently-tagged form of C12 ceramide that displays excitation/emission maxima of 505/540 nm, respectively. BODIPY-C12 Ceramide (d18:1/12:0) Chemical Structure
  25. GC43006 C12 Ceramide (d18:1/12:0) C12 Ceramide (d18:1/12:0) (N-Lauroyl-D-erythro-sphingosine), a naturally occurring ceramide, is formed by hydrolysis of C12 sphingomyelin. C12 Ceramide (d18:1/12:0) Chemical Structure
  26. GC43032 C16 Globotriaosylceramide (d18:1/16:0) C16 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from C16 lactosylceramide. C16 Globotriaosylceramide (d18:1/16:0) Chemical Structure
  27. GC43033 C16 Lactosylceramide (d18:1/16:0) C16 Lactosylceramide is an endogenous bioactive sphingolipid. C16 Lactosylceramide (d18:1/16:0) Chemical Structure
  28. GC43035 C16 Sphingomyelin (d18:1/16:0) Sphingomyelins are complex membrane lipids composed of phosphorylcholine, sphingosine, and an acylated group, such as a fatty acid. C16 Sphingomyelin (d18:1/16:0) Chemical Structure
  29. GC43038 C17 Ceramide (d18:1/17:0) C17 Ceramide is a synthetic ceramide that contains a non-natural C17:0 fatty acid acylated to sphingosine. C17 Ceramide (d18:1/17:0) Chemical Structure
  30. GC43041 C17 Globotriaosylceramide (d18:1/17:0) 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. C17 Globotriaosylceramide (d18:1/17:0) Chemical Structure
  31. GC43049 C18 Globotriaosylceramide (d18:1/18:0) C18 globotriaosylceramide is an endogenous sphingolipid found in mammalian cell membranes that is synthesized from lactosylceramide. C18 Globotriaosylceramide (d18:1/18:0) Chemical Structure
  32. GC43059 C2 Adamantanyl Galactosylceramide (d18:1/2:0)

    C2 Adamantanyl Galactosylceramide (d18:1/2:0) (AdaGalCer) is a bioactive sphingolipid.

     C2 Adamantanyl Galactosylceramide (d18:1/2:0) Chemical Structure
  33. GC43068 C20 Sphingomyelin (d18:1/20:0) C20 Sphingomyelin is a naturally occurring sphingolipid. C20 Sphingomyelin (d18:1/20:0) Chemical Structure
  34. GC40041 Cholesteryl Lignocerate Cholesteryl lignocerate is a cholesterol ester that has been found in human meibum. Cholesteryl Lignocerate Chemical Structure
  35. GC47189 D-erythro/L-threo Lysosphingomyelin (d18:1) A mixture of bioactive sphingolipids D-erythro/L-threo Lysosphingomyelin (d18:1) Chemical Structure
  36. GC47204 D-Fructose-13C6 An internal standard for the quantification of D-fructose D-Fructose-13C6 Chemical Structure
  37. GC49325 Dihydroxyacetone Phosphate (magnesium salt hydrate) A glycerolipid precursor Dihydroxyacetone Phosphate (magnesium salt hydrate) Chemical Structure
  38. GC47286 Eliglustat-d15 (tartrate) An internal standard for the quantification of eliglustat Eliglustat-d15 (tartrate) Chemical Structure
  39. GC18469 Galactosylcerebrosides (bovine) Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with hydroxy and non-hydroxy fatty acids. Galactosylcerebrosides (bovine) Chemical Structure
  40. GC43721 Galactosylcerebrosides (hydroxy) Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid. Galactosylcerebrosides (hydroxy) Chemical Structure
  41. GC43722 Galactosylcerebrosides (non-hydroxy) Galactosylcerebrosides are glycosphingolipids that contain a galactose attached to a ceramide acylated with a hydroxy or non-hydroxy fatty acid. Galactosylcerebrosides (non-hydroxy) Chemical Structure
  42. GC43723 Galactosylsphingosine (d18:1) Galactosylsphingosine (d18:1) (Galactosylsphingosine), a substrate of the galactocerebrosidase (GALC) enzyme, is a potential biomarker for Krabbe disease. Galactosylsphingosine (d18:1) Chemical Structure
  43. GC43727 Ganglioside GD1a mixture (sodium salt)

    Ganglioside GD1a is a sialic acid-containing glycosphingolipid found in brain, erythrocytes, bone marrow, testis, spleen, and liver.

     Ganglioside GD1a mixture (sodium salt) Chemical Structure
  44. GC47392 Ganglioside GM1 Mixture (ovine) (ammonium salt) A mixture of ganglioside GM1 Ganglioside GM1 Mixture (ovine) (ammonium salt) Chemical Structure
  45. GC40016 Ganglioside GM2 Mixture (sodium salt) Ganglioside GM2 is a glycosphingolipid component of cellular membranes, primarily the plasma membrane. Ganglioside GM2 Mixture (sodium salt) Chemical Structure
  46. GC52492 Globotriaosylceramide (hydroxy) (porcine RBC) A sphingolipid Globotriaosylceramide (hydroxy) (porcine RBC) Chemical Structure
  47. GC52491 Globotriaosylceramide (non-hydroxy) (porcine RBC) A sphingolipid Globotriaosylceramide (non-hydroxy) (porcine RBC) Chemical Structure
  48. GC43758 Globotriaosylceramides (hydroxy) (porcine) Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides. Globotriaosylceramides (hydroxy) (porcine) Chemical Structure
  49. GC43760 Globotriaosylceramides (porcine) Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides. Globotriaosylceramides (porcine) Chemical Structure
  50. GC43768 Glucocerebrosides (Gaucher's spleen) Glucocerebrosides are formed by the tethering of glucose to a ceramide by glucosylceramide synthase. Glucocerebrosides (Gaucher's spleen) Chemical Structure
  51. GC52493 Glucosylceramide (bovine buttermilk) A sphingolipid Glucosylceramide (bovine buttermilk) Chemical Structure
  52. GC52219 Heptadecanoyl-L-carnitine (chloride) A long-chain acylcarnitine Heptadecanoyl-L-carnitine (chloride) Chemical Structure
  53. GC52231 Heptadecanoyl-L-carnitine-d3 (chloride) An internal standard for the quantification of heptadecanoyl-L-carnitine Heptadecanoyl-L-carnitine-d3 (chloride) Chemical Structure
  54. GC49678 Heptadecanoyl-L-carnitine-d3 (chloride) MaxSpec® Standard A quantitative analytical standard guaranteed to meet MaxSpec® identity, purity, stability, and concentration specifications Heptadecanoyl-L-carnitine-d3 (chloride) MaxSpec® Standard Chemical Structure
  55. GC49479 Hypoxanthine-d4 An internal standard for the quantification of hypoxanthine Hypoxanthine-d4 Chemical Structure
  56. GC43914 Isobutyryl Coenzyme A (sodium salt) Isobutyryl coenzyme A (isobutyryl-CoA) is a short-chain branched acyl CoA. Isobutyryl Coenzyme A (sodium salt) Chemical Structure
  57. GC52009 Isovaleryl-DL-carnitine-d9 (chloride) An internal standard for the quantification of isovaleryl-DL-carnitine Isovaleryl-DL-carnitine-d9 (chloride) Chemical Structure
  58. GC52323 L-Isoleucine-13C6 An internal standard for the quantification of L-isoleucine L-Isoleucine-13C6 Chemical Structure
  59. GC49782 L-Phenylalanine-13C6 L-Phenylalanine-13C6 Chemical Structure
  60. GC44101 Lyso-Globotriaosylceramide (d18:1) Lyso-globotriaosylceramide is a form of globotriaosylceramide that is lacking the fatty acyl group. Lyso-Globotriaosylceramide (d18:1) Chemical Structure
  61. GC44105 Lysosphingomyelin (d18:1) Sphingomyelins are complex membrane lipids composed of phosphorylcholine, sphingosine, and an acylated group, such as a fatty acid. Lysosphingomyelin (d18:1) Chemical Structure
  62. GC49472 Methylmalonic Acid-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. Methylmalonic Acid-d3 Chemical Structure
  63. GC49246 N-(3-Phenylpropionyl)glycine An acylglycine N-(3-Phenylpropionyl)glycine Chemical Structure
  64. GC49388 N-Butyldeoxynojirimycin-d9 (hydrochloride) An internal standard for the quantification of N-butyldeoxynojirimycin N-Butyldeoxynojirimycin-d9 (hydrochloride) Chemical Structure
  65. GC49240 N-Propionylglycine A glycine-conjugated form of propionic acid N-Propionylglycine Chemical Structure
  66. GC47779 Nitisinone-13C6 An internal standard for the quantification of nitisinone Nitisinone-13C6 Chemical Structure
  67. GC49323 Norcholic Acid A bile acid and derivative of cholic acid Norcholic Acid Chemical Structure
  68. GC46187 Norhyodeoxycholic Acid A synthetic bile acid Norhyodeoxycholic Acid Chemical Structure
  69. GC46192 Octanoic Acid-13C An internal standard for the quantification of octanoic acid Octanoic Acid-13C Chemical Structure
  70. GC45982 Octanoic Acid-d15 An internal standard for the quantification of octanoic acid Octanoic Acid-d15 Chemical Structure
  71. GC45996 Octanoic Acid-d2 An internal standard for the quantification of octanoic acid Octanoic Acid-d2 Chemical Structure
  72. GC45813 Octanoic Acid-d3 An internal standard for the quantification of octanoic acid Octanoic Acid-d3 Chemical Structure
  73. GC44488 Octanoyl Coenzyme A (sodium salt) Octanoyl coenzyme A (octanoyl-CoA) is a medium-chain acyl CoA and a metabolic intermediate in mitochondrial fatty acid β-oxidation. Octanoyl Coenzyme A (sodium salt) Chemical Structure
  74. GC48724 Pregnanetriol A metabolite of 17α-hydroxyprogesterone Pregnanetriol Chemical Structure
  75. GC45981 Pristanic Acid-d3

    An internal standard for the quantification of pristanic acid

     Pristanic Acid-d3 Chemical Structure
  76. GC46215 S-Acetyl-L-glutathione A derivative of glutathione S-Acetyl-L-glutathione Chemical Structure
  77. GC52496 Sulfatide (bovine) (sodium salt) A mixture of isolated bovine sulfatides Sulfatide (bovine) (sodium salt) Chemical Structure
  78. GC44968 Sulfatides (bovine) (sodium salt) Sulfatides are endogenous sulfoglycolipids with various biological activities in the central and peripheral nervous systems, pancreas, and immune system. Sulfatides (bovine) (sodium salt) Chemical Structure
  79. GC40341 Tetrahydro-11-deoxy Cortisol Tetrahydro-11-deoxy cortisol (THS) is the primary urinary metabolite of 11-deoxycortisol. Tetrahydro-11-deoxy Cortisol Chemical Structure
  80. GC45945 Trihydroxycholestanoic Acid A cholic acid intermediate Trihydroxycholestanoic Acid Chemical Structure
  81. GC49328 UDP-α-D-Galactose (sodium salt) A nucleotide sugar UDP-α-D-Galactose (sodium salt) Chemical Structure

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