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Ornithine

Orn
Updated 21/12/2022

What is ornithine?

Ornithine is a non-proteinogenic alpha-amino acid. Within the cell, ornithine is located in the mitochondria and the cytoplasma, and is a central component of the urea cycle, that is involved in the disposal of excess nitrogen (2; Fig. 1).
Ornithine is involved in numerous metabolic processes, including the formation of citrulline, proline and polyamines, and many inborn errors and metabolic diseases (2,3). Health benefits, including enhanced athletic performance and wound healing, have been attributed to increased ornithine intake as supplement, and concentrations in blood and tissues have been linked to chronic diseases such as Alzheimers disease, liver disease and cancer (4).
Method: GC-MS/MS.

Indication(s)

Should be measured together with arginine and citruline.

Specimen, collection and processing

Matrix: EDTA plasma and serum.
Volume: Minimum volume is 50 µL, but 200 µL is optimal and allows reanalysis.
Preparation and stability: The blood sample must be centrifuged and the plasma/serum fraction put on ice, and frozen.

Transportation

Frozen, on dry ice. (for general instruction on transportation, click here)

Reported values, interpretation

Reported values: 30-90 µmol/L
Intraclass correlation coefficient (ICC): NA.

Literature

1. Midttun, Ø., McCann, A., Aarseth, O., Krokeide, M., Kvalheim, G., Meyer, K., and Ueland, P.M. (2016). Combined measurement of 6 fat-soluble vitamins and 26 water-soluble functional vitamin markers and amino acids in 50 μL of serum or plasma by high-throughput mass spectrometry. Anal Chem 88, 10427-436.
2. Barmore, W., Azad, F., & Stone, W. L. (2020). Physiology, Urea Cycle. In StatPearls. StatPearls Publishing.
3. Stone, W. L., Basit, H., & Jaishankar, G. B. (2021). Urea Cycle Disorders. In StatPearls. StatPearls Publishing.
4. Caldwell, R. W., Rodriguez, P. C., Toque, H. A., Narayanan, S. P., & Caldwell, R. B. (2018). Arginase: A Multifaceted Enzyme Important in Health and Disease. Physiol Rev, 98, 641-665.

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Biomarkers included

Amino acids

  • Alanine
  • Arginine
  • Asparagine
  • Aspartic acid
  • Glutamic acid
  • Glutamine
  • Glycine
  • Histidine
  • Isoleucine
  • Kynurenine
  • Leucine
  • Lysine
  • Methionine
  • Ornithine
  • Phenylalanine
  • Proline
  • Sarcosine
  • Serine
  • Threonine
  • Total cysteine
  • Tryptophan
  • Tyrosine
  • Valine

Amino acids and catabolites

  • 2-Aminoadipic acid
  • 2-Hydroxybutyrate
  • 3-Hydroxysiobutyrate
  • a-Hydroxyglutaric acid
  • B-Alanine
  • B-Aminoisobutyrate
  • B-Hydroxy B-methylbutyric acid
  • Phenylacetylglutamine

Acylcarnities

  • BB Butyrobetaine
  • C0 Carnitine
  • C2 Acetylcarnitine
  • C3 Propionylcarnitine
  • C3-DC Malonylcarnitine
  • C4 Butyrylcarnitine
  • C4-OH Hydroxybutyrylcarnitine
  • C4-DC Succinylcarnitine
  • iC5 Isovalerylcarnitine
  • C5-DC Glutarylcarnitine
  • C5:1 Tiglylcarnitine
  • C6 Hexanoylcarnitine
  • C8 Octanoylcarnitine
  • C10 Decanoylcarnitine
  • C12 Dodecanoylcarnitine
  • C14 Tetradecanoylcarnitine
  • C14-OH Hydroxytetradecanoylcarnitine
  • C16 Hexadecanoylcarnitine
  • C16-OH Hydroxyhexadecanoylcarnitine
  • C18 Octadecanoylcarnitine
  • C18-OH Hydroxyoctadecanoylcarnitine
  • C18:1 Octadecenoylcarnitine
  • C18:2 Octadecadienylcarnitine

TCA metabolites

  • a-Ketoglutarate
  • Citrate
  • Fumarate
  • Isocitrate
  • Lactate
  • Malate
  • Pyruvate

Ketone bodies

  • 3-Hydroxybutyrate
  • Acetoacetate

AGEs

Biomarkers included

Kynurenines

 

Proteins

  • C-Reactive protein
  • Calprotectin
  • Serum Amyloid A

Others

  • Kynurenine/tryptophan ratio
  • Neopterin
  • PAr index (PLP, PL, PA)
Biomarkers included

SCFAs

  • Acetate
  • Butyrate
  • Formate
  • Isobutyrate
  • Isovalerate
  • Propionate
  • Valerate
  • α-Methylbutyrate

Indoles

  • 3-Indoxyl sulfate
  • Imidazole propionate
  • Indole-3-acetamide
  • Indole-3-acetate
  • Indole-3-aldehyde
  • Indole-3-lactate
  • Indole-3-propionate

Choline metabolites

  • Choline
  • Betaine
  • DMG
  • TMAO

Amino acid derived

  • Phenylacetylglutamine
Biomarkers included

B-vitamins, functional markers, and methyl donors

  • 4-Pyridoxic acid
  • Betaine
  • Choline
  • Cobalamin
  • Flavin mononucleotide
  • Folate
  • Methylmalonic acid
  • N1-methylnicotinamide
  • Nicotinamide
  • Nicotinic acid
  • Pyridoxal
  • Pyridoxal 5-phosphate
  • Pyridoxine
  • Riboflavin
  • Thiamine
  • Thiamine monophosphate
  • Total homocysteine

Fat-soluble vitamins

  • 25-hydroxy vitamin D2
  • 25-hydroxy vitamin D3
  • α-Tocopherol (Vit. E)
  • All-trans retinol (Vit. A)
  • Phylloquinone (Vit. K1)
  • y-Tocopherol (Vit. E)

Essential amino acids

  • Histidine
  • Isoleucine
  • Leucine
  • Lysine
  • Methionine
  • Phenylalanine
  • Threonine
  • Tryptophan
  • Valine

Meat and fish intake

  • 1-Methylhistidine
  • 3-Methylhistidine
  • B-Alanine
  • Creatine
  • Creatinine
  • TMAO

Tobacco use and coffee intake

  • Cotinine
  • Trans-3-hydroxycotinine
  • Trigonelline

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