TABLE 4

Very long-chain acylcarnitines and diseases with altered very long-chain acylcarnitine levels in blood (serum, plasma, dried blood spots) or urine

Acylcarnitine (Fatty Acid Moiety)SampleIncreased/ decreasedDisease with Altered Acylcarnitine Concentration (Reference)
Behenoylcarnitine (C22:0)BloodDecreasedAcute cerebral infarction (Zhang et al., 2017b)
UrineIncreasedZellweger syndrome (Duranti et al., 2008); infantile Refsum disease (Duranti et al., 2008); D-bifunctional protein deficiency (Duranti et al., 2008)
Lignoceroylcarnitine (C24:0)BloodIncreasedType 2 diabetes (Zhang et al., 2014)
DecreasedCardiovascular diseases in type 2 diabetes (Zhao et al., 2020)
UrineIncreasedZellweger syndrome (Duranti et al., 2008); infantile Refsum disease (Duranti et al., 2008); type 2 diabetes (Zhang et al., 2014)
Cerotoylcarnitine (C26:0)BloodIncreasedZellweger syndrome (Klouwer et al., 2017), X-linked adrenoleukodystrophy (van de Beek et al., 2016), cardiovascular mortality in chronic kidney disease (Kalim et al., 2013)
UrineIncreasedZellweger syndrome (Duranti et al., 2008); infantile Refsum disease (Duranti et al., 2008)