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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. GC26265 α-Hydroxyglutaric acid disodium

    2-Hydroxyglutarate disodium; 2-Hydroxyglutaric acid disodium; 2-Hydroxypentanedioic acid disodium

    α-Hydroxyglutaric acid (2-Hydroxyglutarate) disodium 是戊二酸的 α-羟基酸形式。
  3. GC91083 Tiopronin-Cysteine Disulfide

    α-Mercaptopropionylglycine-Cysteine Disulfide

    一种硫普罗宁的代谢产物。
  4. GC52496 Sulfatide (bovine) (sodium salt)

    Galactosylceramide I3-sulfate, Ganglioside SM4, I3SO3-GalCer, SM4, Sulfated Galactocerebroside, 3-O-Sulfogalactosylceramide

     A mixture of isolated bovine sulfatides
  5. GC52493 Glucosylceramide (bovine buttermilk)

    GluCers (buttermilk), Glucocerebrosides (buttermilk)

     A sphingolipid
  6. GC52492 Globotriaosylceramide (hydroxy) (porcine RBC)

    Ceramide Trihexosides (bottom spot) (porcine)

     A sphingolipid
  7. GC52491 Globotriaosylceramide (non-hydroxy) (porcine RBC)

    Ceramide Trihexosides (top spot) (porcine)

     A sphingolipid
  8. GC52404 γ-Glu-Phe (trifluoroacetate salt)

    γ-Glutamylphenylalanine

     A dipeptide with metabolism-altering activity
  9. GC52323 L-Isoleucine-13C6

    L-(+)-Isoleucine-13C6

     An internal standard for the quantification of L-isoleucine
  10. 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
  11. GC52219 Heptadecanoyl-L-carnitine (chloride)

    CAR 17:0, C17:0 Carnitine, L-Carnitine heptadecanoyl ester, L-Heptadecanoylcarnitine

     A long-chain acylcarnitine
  12. GC52122 2’-Deoxyadenosine-5’-diphosphate (sodium salt)

    2'-脱氧腺苷-5'-二磷酸三钠

     A nucleotide diphosphate
  13. GC52083 21-Deoxycortisone

    17α-羟基孕甾-4-烯-3,11,20-三酮

     A corticosteroid metabolite of 11-keto progesterone
  14. 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
  15. GC19941 DL-Leucic Acid
  16. GC49840 2-Methylbutyrylglycine

    N-(2-甲基-1-氧代丁基)-甘氨酸

     A metabolite of isoleucine
  17. GC49782 L-Phenylalanine-13C6

    L-苯丙氨酸-13C6,(S)-2-Amino-3-phenylpropionic acid-13C6

     An internal standard for the quantification of phenylalanine
  18. GC19926 Methyl malonic acid

    丙二酸二甲酯

     A dicarboxylic acid and byproduct of propionate catabolism
  19. GC49678 Heptadecanoyl-L-carnitine-d3 (chloride) MaxSpec® Standard

    CAR 17:0-d3, C17:0 Carnitine-d3, L-Carnitine heptadecanoyl ester-d3, L-Heptadecanoylcarnitine-d3

     A quantitative analytical standard guaranteed to meet MaxSpec® identity, purity, stability, and concentration specifications
  20. GC49499 5(Z)-Dodecenoic Acid

    顺-5-十二碳烯酸

     A medium-chain unsaturated fatty acid
  21. GC49479 Hypoxanthine-d4

    次黄嘌呤

     An internal standard for the quantification of hypoxanthine
  22. GC49472 Methylmalonic Acid-d3

    甲基(D3)丙二酸,Methylpropanedioic acid-d3; Methylmalonate-d3

     An internal standard for the quantification of methylmalonic acid
  23. 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
  24. GC49388 N-Butyldeoxynojirimycin-d9 (hydrochloride)

    [2H9]-N-丁基脱氧野尻霉素盐酸盐,N-Butyldeoxynojirimycin-d9 hydrochloride; NB-DNJ-d9 hydrochloride; OGT 918-d9 hydrochloride

     An internal standard for the quantification of N-butyldeoxynojirimycin
  25. GC49328 UDP-α-D-Galactose (sodium salt)

    UDP-Α-D半乳糖二钠盐

     A nucleotide sugar
  26. GC49325 Dihydroxyacetone Phosphate (magnesium salt hydrate)

    DHAP, Glycerone Phosphate

     甘油脂前体
  27. GC49323 Norcholic Acid

    23-Norcholic Acid

     A bile acid and derivative of cholic acid
  28. GC49246 N-(3-Phenylpropionyl)glycine

    N-(3-苯基丙酰基)甘氨酸

     An acylglycine
  29. GC49240 N-Propionylglycine

    丙甘氨酸

     A glycine-conjugated form of propionic acid
  30. GC49149 3-Methylglutaconic Acid

    3-甲基戊烯二酸

     A branched-chain organic acid
  31. GC48724 Pregnanetriol

    孕三醇

     A metabolite of 17α-hydroxyprogesterone
  32. GC48345 Lyso-Monosialoganglioside GM2 (ammonium salt)

    Lysoganglioside GM2, Lyso-GM2, lyso-Monosialoganglioside GM2

     A sphingolipid
  33. GC47779 Nitisinone-13C6

    尼替西农-13C6

     An internal standard for the quantification of nitisinone
  34. GC47393 Ganglioside GM1 Mixture (porcine brain) (ammonium salt)

    GM1, Monosialoganglioside GM1

     A sphingolipid
  35. GC47392 Ganglioside GM1 Mixture (ovine) (ammonium salt)

    GM1, Monosialoganglioside GM1

     A sphingolipid
  36. GC47286 Eliglustat-d15 (tartrate)

    Genz 99067-d15 tartrate

     An internal standard for the quantification of eliglustat
  37. GC47204 D-Fructose-13C6

    D-果糖 13C6

     An internal standard for the quantification of D-fructose
  38. 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
  39. GC47115 Coenzyme B12 (hydrate)

    Adenosylcobalamin, AdoCbl, Cobamamide, Dibenzcozamide, LM-176

     A neuropeptide with diverse biological activities
  40. 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
  41. 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
  42. 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
  43. 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
  44. GC46735 7-Biopterin

    Primapterin, L-Primapterin

     A pterin
  45. GC46584 3-Aminoisobutyric Acid (sodium salt)

    β-Aminoisobutyric Acid, BAIBA

     A neuropeptide with diverse biological activities
  46. GC46540 2'-Deoxyadenosine-5'-triphosphate (sodium salt hydrate) A purine nucleotide
  47. GC46243 24-dehydro Cholesterol-d6

    Desmosterol-d6

     An internal standard for the quantification of 24-dehydro cholesterol
  48. GC46242 7-dehydro Cholesterol-d7

    7-去氢胆固醇 d7

     An internal standard for the quantification of 7-dehydro cholesterol
  49. GC46215 S-Acetyl-L-glutathione

    S-乙酰-L-谷胱甘肽

     A derivative of glutathione
  50. GC46192 Octanoic Acid-13C

    辛酸-1-13C,Caprylic acid-13C

     An internal standard for the quantification of octanoic acid
  51. GC46187 Norhyodeoxycholic Acid

    去甲去氧胆酸

     A synthetic bile acid

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