Search Site
  • 0
    My Cart

    Click Here to Find How Many Items You Have Added:)

qq在线咨询
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. 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
  3. GC46068 3β-OH-7-Oxocholenic Acid

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

     A bile acid
  4. GC45996 Octanoic Acid-d2

    2,2-氘代辛酸(D2),Caprylic acid-d2

     An internal standard for the quantification of octanoic acid
  5. GC45982 Octanoic Acid-d15

    氘代辛酸(D15),Caprylic acid-d15

     An internal standard for the quantification of octanoic acid
  6. GC45981 Pristanic Acid-d3

    降植烷酸

    An internal standard for the quantification of pristanic acid
  7. GC45945 Trihydroxycholestanoic Acid

    粪甾烷酸,Trihydroxycoprostanic Acid

     A cholic acid intermediate
  8. GC45813 Octanoic Acid-d3

    8,8,8-氘代辛酸(D3),Caprylic acid-d3

     An internal standard for the quantification of octanoic acid
  9. GC45661 C16 Sphingomyelin-d9 (d18:1/16:0-d9)

    Palmitoyl Sphingomyelin-d9

     具有多种生物活性的神经肽
  10. GC44968 Sulfatides (bovine) (sodium salt) Sulfatides are endogenous sulfoglycolipids with various biological activities in the central and peripheral nervous systems, pancreas, and immune system.
  11. GC44639 phytanoyl-Coenzyme A (triethylammonium salt)

    Phytanic Acid Coenzyme A Ester (triethylammonium salt), phytanoyl-CoA (triethylammonium salt)

     A derivative of phytanic acid
  12. GC44488 Octanoyl Coenzyme A (sodium salt)

    Octanoyl-CoA

     A medium-chain acyl CoA
  13. 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)

     A bioactive sphingolipid
  14. GC44101 Lyso-Globotriaosylceramide (d18:1)

    Globotriaosylsphingosine (d18:1), Lyso-Ceramide Trihexoside, Lyso-Gb3

     A globotriaosylceramide lacking the fatty acyl group
  15. GC43914 Isobutyryl Coenzyme A (sodium salt)

    Isobutyryl-CoA

     A short-chain branched acyl CoA
  16. GC43768 Glucocerebrosides (Gaucher's spleen)

    Ganglioside GL1a, Gaucher cerebroside, GluCers (Gaucher's spleen), Glucocerebrosides, Glucosylceramides (Gaucher's spleen)

     A sphingolipid
  17. GC43760 Globotriaosylceramides (porcine)

    球形三酰神经

     A sphingolipid
  18. GC43758 Globotriaosylceramides (hydroxy) (porcine) Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides.
  19. GC43727 Ganglioside GD1a mixture (sodium salt)

    双唾液酸神经节苷酯GD1A,Ganglioside B1

     A mixture of ganglioside GD1a
  20. GC43723 Galactosylsphingosine (d18:1)

    神经鞘氨醇半乳糖苷,Galactosylsphingosine

     A bioactive sphingolipid
  21. GC43722 Galactosylcerebrosides (non-hydroxy)

    猪脑苷脂

     A sphingolipid
  22. GC43721 Galactosylcerebrosides (hydroxy)

    Cerebrosides; Phrenosin (bottom spot)

     A mixture of isolated bovine hydroxy galactosylceramides
  23. GC43486 DL-Cystathionine (hydrochloride)

    DL-Allocystathionine

     An intermediate in cysteine synthesis
  24. GC43068 C20 Sphingomyelin (d18:1/20:0)

    C20鞘磷脂(D18:1/20:0)

     A sphingolipid
  25. 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。
  26. 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
  27. GC43041 C17 Globotriaosylceramide (d18:1/17:0)

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

     A sphingolipid
  28. GC43038 C17 Ceramide (d18:1/17:0)

    Cer(d18:1/17:0), Ceramide (d18:1/17:0), N-heptadecanoyl-D-erythro-Sphingosine

     A synthetic ceramide
  29. GC43035 C16 Sphingomyelin (d18:1/16:0)

    N-棕榈酰-D-赤型鞘氨酰磷酸胆碱

     A sphingolipid
  30. 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
  31. 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
  32. 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
  33. 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
  34. GC42695 Acetoacetyl Coenzyme A (sodium salt hydrate)

    Acetoacetyl-CoA

     A precursor to HMG-CoA
  35. 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
  36. 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
  37. GC42292 3-Hydroxypropionic Acid (sodium salt)

    Sodium β-hydroxypropionate

     A building block in the synthesis of a variety of compounds
  38. GC42051 1-β-D-Glucosylsphingosine (d18:1)

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

     A lysolipid derivative of glucocerebroside
  39. 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
  40. GC41614 24-dehydro Cholesterol

    胆固醇EP杂质B (Desmosterol)

     An immediate precursor of cholesterol
  41. GC41582 4(Z),7(Z)-Decadienoic Acid A PUFA
  42. 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
  43. GC40341 Tetrahydro-11-deoxy Cortisol

    四氢化-11-脱氧皮质醇

     A metabolite of 11-deoxycortisol
  44. 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
  45. GC40041 Cholesteryl Lignocerate

    24:0 CE, 24:0 Cholesterol ester

     A cholesterol ester
  46. GC40016 Ganglioside GM2 Mixture (sodium salt)

    单唾液酸神经节苷脂GM2; GM2; Monosialoganglioside GM2

     A mixture of ganglioside GM2
  47. GC18609 1-Deoxysphinganine (m18:0)

    ES-285

     An atypical sphingolipid
  48. GC18469 Galactosylcerebrosides (bovine)

    半乳糖苷神经酰胺

     A sphingolipid

Items 51 to 97 of 97 total

per page
  2. 1
  3. 2

Set Descending Direction