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Table 1 Amino acids in blood, urine and CSF

From: Novel variants in a patient with late-onset hyperprolinemia type II: diagnostic key for status epilepticus and lactic acidosis

Amino acidblood μmol/lreference μmol/lurine μmol/g Creareference μmol/g CreaCSF mg/dlReference mg/dl
Alanine685205–508929.0< 7003219–60
Arginine11340–140< 9.0< 1501811–32
Asparagine9039–7959.0< 500105–20
Aspartic acid51< 35352.0< 100< 3< 3
Cysteine  31< 2004< 3
Glutamine284470–758383.0< 800466380–1348
Glutamine acid42028–9225.< 200< 3< 4
Glycine710120–38719,664.0< 250012< 35
Histidine11830–120781.0< 1600169–28
Hydroxyproline1395< 100
Isoleucine6835–10019.0< 100< 3< 17
Lysine21782–260401.0< 2502113–42
Methionine236–4056.0< 100< 3< 10
Phenylalanine12037–115160< 15077–21
Proline308590–34246,531< 100104< 6
Serine22667–19312.0< 8003119–40
Threonine15275–19425< 5003623–57
Tryptophan50.034–9015.0< 150< 3< 6
Tyrosine86.021–107386.0< 200135–17
Valine198116–31777.0< 120911–40
  1. Values were obtained in a fasted state in the morning.
  2. Significant data are set in Bold