Acylcarnitines

Acylcarnitines are intermediates of fatty acid and amino acid oxidation found in tissues and body fluids. Acylcarnitines are important diagnostic markers for inherited diseases of peroxisomal and mitochondrial oxidation processes and abnormalities in specific acylcarnitine concentrations are used in the identification of carnitine deficiency and diagnosis of fatty acids oxidation defects and organic acidurias such as carnitine-acylcarnitine translocase deficiency (CACTD) or Isobutyryl-CoA dehydrogenase deficiency. Measuring different acylcarnitines can be used to detect more than 40 different inborn errors of metabolism. If these diseases are not diagnosed, the metabolic disorders can already lead to severe irreversible harm to newborns within their first few days of life. Newborn screening programs aim to detect congenital metabolic disorders early on in infants before they become symptomatic. Liquid chromatography-tandem mass spectrometry (LC-MS/MS) has greatly increased the screening possibilities in newborn screening programs and has been established as an efficient and robust methodology for analyzing acyl-L-carnitines. Detection by MS provides the required sensitivity and direct infusion may be sufficient to detect several disorders with a single injection. For more detailed analysis and for measuring acylcarnitine isomers that are closely related, separation by liquid chromatography before detection is important.

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A-148 Acylcarnitines IS Mix 1 (C0, C2) solution 1 mg/mL in methanol (each analyte), certified reference material, ampule of 1 mL, Cerilliant®
A-141 Acylcarnitines Mix 1 (C0,C2) hydrochloride solution 10 mg/mL in methanol (each analyte as free base), certified reference material, ampule of 1 mL, Cerilliant®
A-144 Acylcarnitines Mix 2 (C4,iC4,C5,iC5,C6,C8,C10,C12,C14,C18) solution 100-200 μg/mL (90:10 Methanol: 1% aqueous formic acid), certified reference material, ampule of 1 mL, Cerilliant® New
A-176 Glutaryl-L-Carnitine lithium salt solution 100 μg/mL (90:10 Methanol: 1% aqueous formic acid), certified reference material, ampule of 1 mL, Cerilliant® New
A-181 Glutaryl-L-Carnitine-(N-Methyl-D3) lithium salt solution 100 μg/mL (90:10 Methanol: 1% aqueous formic acid), certified reference material, ampule of 1 mL, Cerilliant® New
A-162 Palmitoyl-L-Carnitine solution 5 mg/mL (90:10 Methanol:DMSO), certified reference material, ampule of 1 mL, Cerilliant®
A-164 Palmitoyl-L-Carnitine (N-Methyl-D3) solution 500 μg/mL (90:10 Methanol:DMSO), certified reference material, ampule of 1 mL, Cerilliant®
A-161 Propionyl-L-carnitine solution 5 mg/mL (90:10 Methanol:DMSO), certified reference material, ampule of 1 mL, Cerilliant®
A-163 Propionyl-L-Carnitine-(N- Methyl-D3) solution 500 μg/mL (90:10 Methanol:DMSO), certified reference material, ampule of 1 mL, Cerilliant®