DL-Carnitine HCl - CAS 461-05-2
Catalog number:
461-05-2
Category:
Inhibitor
Not Intended for Therapeutic Use. For research use only.
Molecular Formula:
C7H15NO3.HCl
Molecular Weight:
197.66
COA:
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Description:
DL-Carnitine HCl is a quaternary ammonium compound biosynthesized from the amino acids lysine and methionine.
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Purity:
>98%
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1.Riboflavin-responsive lipid-storage myopathy and glutaric aciduria type II of early adult onset.
de Visser M, Scholte HR, Schutgens RB, Bolhuis PA, Luyt-Houwen IE, Vaandrager-Verduin MH, Veder HA, Oey PL. Neurology. 1986 Mar;36(3):367-72.
A 17-year-old girl with progressive lipid-storage myopathy for 2 years had low muscle carnitine levels. There was no therapeutic response to prednisone and DL-carnitine-HCl. Chemical findings indicated glutaric aciduria type II. Riboflavin therapy and a fat-restricted, carbohydrate-enriched diet resulted in dramatic improvement. Low carnitine concentrations in plasma and muscle were observed in three asymptomatic sisters who had normal urinary excretion patterns. There was impaired mitochondrial beta-oxidation in cultured skin fibroblasts from the index patient and all three siblings.
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CAS 461-05-2 DL-Carnitine HCl

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