Propionylcarnitine is an ester metabolite generated by L-carnitine and propionyl-CoA under the catalysis of carnitine acyltransferase[1]. Elevated levels of Propionylcarnitine in serum are an indirect biomarker of vitamin B12 deficiency, because B12 deficiency inhibits the conversion of propionyl-CoA to succinyl-CoA, leading to accumulation of Propionylcarnitine[2]. Propionylcarnitine can act as a superoxide scavenger, antioxidant, and DNA cleavage protector[3]. Propionylcarnitine can alleviate oxidative stress damage to red blood cells in β-thalassemia[4].
References:
[1] Roseiro L C, Santos C. Carnitines (including l-carnitine, acetyl-carnitine, and proprionyl-carnitine)[M]//Nonvitamin and nonmineral nutritional supplements. Academic Press, 2019: 45-52.
[2] Hannibal L, Lysne V, Bjørke-Monsen A L, et al. Biomarkers and algorithms for the diagnosis of vitamin B12 deficiency[J]. Frontiers in molecular biosciences, 2016, 3: 27.
[3] Vanella A, Russo A, Acquaviva R, et al. L-propionyl-carnitine as superoxide scavenger, antioxidant, and DNA cleavage protector[J]. Cell biology and toxicology, 2000, 16: 99-104.
[4] Malaguarnera M, Vacante M, Giordano M, et al. L-carnitine supplementation improves hematological pattern in patients affected by HCV treated with Peg interferon-α 2b plus ribavirin[J]. World journal of gastroenterology: WJG, 2011, 17(39): 4414.
Propionylcarnitine是L-肉碱与丙酰辅酶A（Propionyl-CoA）在肉碱酰基转移酶（Carnitine Acyltransferase）催化下生成的酯类代谢物[1]。血清中 Propionylcarnitine的升高是维生素B12缺乏的间接生物标志物，因为B12缺乏会抑制丙酰辅酶A向琥珀酰辅酶A的转化，导致丙酰肉碱蓄积[2]。Propionylcarnitine能够作为超氧化物清除剂、抗氧化剂和DNA切割保护剂[3]。Propionylcarnitine在β-地中海贫血中能够缓解红细胞的氧化应激损伤[4]。