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

Inborn Errors of Metabolism(先天性代谢缺陷病)

  1. Cat.No. 产品名称 Information
  2. GC52404 γ-Glu-Phe (trifluoroacetate salt)

    γ-Glutamylphenylalanine

     A dipeptide with metabolism-altering activity
  3. GC42051 1-β-D-Glucosylsphingosine (d18:1)

    Glucosyl-C18-Sphingosine, Glucosylsphingosine (d18:1), 1-β-D-Glucosylsphingosine (synthetic)

     A lysolipid derivative of glucocerebroside
  4. GC18609 1-Deoxysphinganine (m18:0)

    ES-285

     An atypical sphingolipid
  5. 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)

     An internal standard for the quantification of C16 sphingomyelin
  6. GC52122 2’-Deoxyadenosine-5’-diphosphate (sodium salt)

    2'-脱氧腺苷-5'-二磷酸三钠

     A nucleotide diphosphate
  7. GC46540 2'-Deoxyadenosine-5'-triphosphate (sodium salt hydrate) A purine nucleotide
  8. GC49840 2-Methylbutyrylglycine

    N-(2-甲基-1-氧代丁基)-甘氨酸

     A metabolite of isoleucine
  9. GC52083 21-Deoxycortisone

    17α-羟基孕甾-4-烯-3,11,20-三酮

     A corticosteroid metabolite of 11-keto progesterone
  10. GC41614 24-dehydro Cholesterol

    胆固醇EP杂质B (Desmosterol)

     An immediate precursor of cholesterol
  11. GC46243 24-dehydro Cholesterol-d6

    Desmosterol-d6

     An internal standard for the quantification of 24-dehydro cholesterol
  12. GC46068 3β-OH-7-Oxocholenic Acid

    3β-hydroxy-7-oxo-5-Cholenoic Acid, 3β-hydroxy-7-Oxochol-5-enoic Acid

     A bile acid
  13. GC46584 3-Aminoisobutyric Acid (sodium salt)

    β-Aminoisobutyric Acid, BAIBA

     A neuropeptide with diverse biological activities
  14. GC42292 3-Hydroxypropionic Acid (sodium salt)

    Sodium β-hydroxypropionate

     A building block in the synthesis of a variety of compounds
  15. GC49149 3-Methylglutaconic Acid

    3-甲基戊烯二酸

     A branched-chain organic acid
  16. GC41582 4(Z),7(Z)-Decadienoic Acid A PUFA
  17. GC42445 4-Methylumbelliferyl β-D-Galactopyranoside-6-sulfate (sodium salt)

    4-甲基香豆素基-Β-D-吡喃半乳糖苷-6-硫酸钠盐

     A fluorogenic substrate of GALNS
  18. GC42442 4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (sodium salt)

    4-甲基香豆素-2-磺胺-2-去氧基-Α-D-葡糖苷酸钠盐

     A fluorogenic substrate of heparin sulphamidase
  19. GC40510 4-Methylumbelliferyl-α-D-Galactopyranoside

    4-甲基伞形酮-α-D-吡喃半乳糖苷; 4MU-α-Gal

     A fluorogenic substrate of α-galactosidase
  20. GC19852 4-Methylumbelliferyl-α-D-glucopyranoside

    7-(α-D-吡喃葡糖氧基)-4-甲基-2H-1-苯并吡喃-2-酮

     A fluorogenic substrate for α-glucosidase
  21. GC11870 4-Methylumbelliferyl-β-D-Glucopyranoside

    4-甲基伞形酮酰-Β-D-吡喃葡糖酸苷,4-MU-GLU;4-MU-β-Gluc;4-MUG;MU-GLU

     A fluorescent substrate of β-glucosidase and β-glucocerebrosidase
  22. GC49499 5(Z)-Dodecenoic Acid

    顺-5-十二碳烯酸

     A medium-chain unsaturated fatty acid
  23. GC46735 7-Biopterin

    Primapterin, L-Primapterin

     A pterin
  24. GC46242 7-dehydro Cholesterol-d7

    7-去氢胆固醇 d7

     An internal standard for the quantification of 7-dehydro cholesterol
  25. GC42695 Acetoacetyl Coenzyme A (sodium salt hydrate)

    Acetoacetyl-CoA

     A precursor to HMG-CoA
  26. GC46083 Adenylosuccinic Acid

    腺苷酸基琥珀酸,Aspartyl Adenylate

    腺苷琥珀酸是一种嘌呤核苷酸，也是嘌呤核苷酸循环中的中间产物
  27. 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

     A fluorescently-tagged C12 ceramide
  28. GC43006 C12 Ceramide (d18:1/12:0)

    N-月桂酰-D-鞘胺醇,N-Lauroyl-D-erythro-sphingosine; N-Laurylsphingosine

     A naturally occurring ceramide
  29. GC43032 C16 Globotriaosylceramide (d18:1/16:0)

    C16 Ceramide Trihexoside (d18:1/16:0), Gb3 (d18:1/16:0), N-Hexadecanoyl-ceramide trihexoside

     A sphingolipid
  30. 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
  31. 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)

     A bioactive sphingolipid
  32. 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
  33. GC43035 C16 Sphingomyelin (d18:1/16:0)

    N-棕榈酰-D-赤型鞘氨酰磷酸胆碱

     A sphingolipid
  34. GC45661 C16 Sphingomyelin-d9 (d18:1/16:0-d9)

    Palmitoyl Sphingomyelin-d9

     具有多种生物活性的神经肽
  35. 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)是一种合成神经酰胺，含有酰化为鞘氨醇的非天然 C17:0脂肪酸。
  36. GC43041 C17 Globotriaosylceramide (d18:1/17:0)

    C17 Ceramide Trihexoside (d18:1/17:0), N-Heptadecanoyl Ceramide Trihexoside

     A sphingolipid
  37. 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
  38. GC43049 C18 Globotriaosylceramide (d18:1/18:0)

    C18 Ceramide Trihexoside (d18:1/18:0), Gb3(d18:1/18:0), N-Octadecanoyl-ceramide trihexoside

     A sphingolipid
  39. 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
  40. GC43059 C2 Adamantanyl Galactosylceramide (d18:1/2:0) C2 金刚烷基半乳糖神经酰胺 (d18:1/2:0) (AdaGalCer) 是一种具有生物活性的鞘脂。
  41. GC43068 C20 Sphingomyelin (d18:1/20:0)

    C20鞘磷脂(D18:1/20:0)

     A sphingolipid
  42. GC40041 Cholesteryl Lignocerate

    24:0 CE, 24:0 Cholesterol ester

     A cholesterol ester
  43. GC47115 Coenzyme B12 (hydrate)

    Adenosylcobalamin, AdoCbl, Cobamamide, Dibenzcozamide, LM-176

     A neuropeptide with diverse biological activities
  44. 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
  45. GC47204 D-Fructose-13C6

    D-果糖 13C6

     An internal standard for the quantification of D-fructose
  46. GC49325 Dihydroxyacetone Phosphate (magnesium salt hydrate)

    DHAP, Glycerone Phosphate

     甘油脂前体
  47. GC43486 DL-Cystathionine (hydrochloride)

    DL-Allocystathionine

     An intermediate in cysteine synthesis
  48. GC19941 DL-Leucic Acid
  49. GC47286 Eliglustat-d15 (tartrate)

    Genz 99067-d15 tartrate

     An internal standard for the quantification of eliglustat
  50. GC18469 Galactosylcerebrosides (bovine)

    半乳糖苷神经酰胺

     A sphingolipid
  51. GC43721 Galactosylcerebrosides (hydroxy)

    Cerebrosides; Phrenosin (bottom spot)

     A mixture of isolated bovine hydroxy galactosylceramides

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