TABLE 3

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

Acylcarnitine (Fatty Acid Moiety)SampleIncreased/ decreasedDisease with Altered Acylcarnitine Concentration
Myristoylcarnitine (14:0)BloodIncreasedVery long-chain acyl-CoA dehydrogenase deficiency (Costa et al., 1997; Peng et al., 2013); carnitine/acylcarnitine translocase deficiency (Wasant et al., 2002); carnitine palmitoyl transferase 2 deficiency (Minkler et al., 2005); glutaric acidemia type 2 (Peng et al., 2013); sleep deprivation (Yoon et al., 2019); type 2 diabetes (Adams et al., 2009); cardiovascular diseases in type 2 diabetes (Zhao et al., 2020); cardiovascular mortality in chronic kidney disease (Kalim et al., 2013)
DecreasedCeliac disease (Bene et al., 2005); chronic fatigue syndrome (Reuter and Evans, 2011)
UrineIncreasedUterine fibroids (Wang et al., 2020)
Palmitoylcarnitine (C16:0)BloodIncreasedVery long-chain acyl-CoA dehydrogenase deficiency (Costa et al., 1997); sleep deprivation (Yoon et al., 2019); carnitine palmitoyl transferase 2 deficiency (Minkler et al., 2005); carnitine/acylcarnitine translocase deficiency (Wasant et al., 2002); type 2 diabetes (Mihalik et al., 2010; Zhang et al., 2014; Qiu et al., 2016); nonalcoholic fatty liver disease (Chen et al., 2016); obesity (Mihalik et al., 2010); pulmonary arterial hypertension (Brittain et al., 2016); chronic heart failure (Ueland et al., 2013); cardiovascular mortality in chronic kidney disease (Kalim et al., 2013); diastolic heart failure (Zordoky et al., 2015; Hunter et al., 2016); systolic heart failure (Hunter et al., 2016); increased all-cause mortality and hospitalization in heart failure patients (Ahmad et al., 2016)
DecreasedIntracerebral hemorrhage (Zhang et al., 2017b); carnitine palmitoyltransferase 1A deficiency (Fingerhut et al., 2001); psoriasis (Chen et al., 2021)
FecesIncreasedCirrhosis (Huang et al., 2013)
Stearoylcarnitine (C18:0)BloodIncreasedCarnitine/acylcarnitine translocase deficiency (Wasant et al., 2002); chronic fatigue syndrome (Reuter and Evans, 2011); pulmonary arterial hypertension (Brittain et al., 2016); carnitine palmitoyltransferase 2 deficiency (Minkler et al., 2005); cardiovascular mortality in chronic kidney disease (Kalim et al., 2013); diastolic heart failure (Hunter et al., 2016); systolic heart failure (Hunter et al., 2016)
DecreasedIntracerebral hemorrhage (Zhang et al., 2017b); carnitine palmitoyltransferase 1A deficiency (Fingerhut et al., 2001)