Succinyl-coenzyme A (succinyl-CoA) is an intermediate in the citric acid cycle.[1] It is converted to succinate, which is a metabolic intermediate with various biological activities. Succinyl-CoA is required, with glycine, to form δ-aminolevulinic acid in the first step of porphyrin and heme synthesis.[2] Succinyl-CoA deficiency, caused by vitamin B12 deficiency can disrupt heme and energy production, leading to neuromotor dysfunction.[3]
琥珀酰辅酶A（琥珀酰-CoA）是三羧酸循环中的中间产物[1]。它转化为丙酸，后者是具有各种生物活性的代谢中间体。琥珀酰-CoA与甘氨酸一起参与卟吩和血红素合成中的第一步反应，形成δ-氨基腺嘌呤酸[2]。由于维生素B12缺乏引起的琥珀酰-CoA缺乏可能会破坏血红素和能量生产，导致神经运动障碍[3]。
Reference:
[1]. Meister, A. ACYL phosphates as enzyme-bound intermediates in the biosynthesis of glutamine, glutathione, succinyl, coenzyme A, and cabamyl phosphate. Trans. N. Y. Acad. Sci. 30(8), 1157-1180 (1968).
[2]. Bonkovsky, H.L., Guo, J.-T., Hou, W., et al. Porphyrin and heme metabolism and the porphyrias. Compr Physiol 3(1), 365-401 (2013).
[3]. Bicakci, Z. Growth retardation, general hypotonia, and loss of acquired neuromotor skills in the infants of mothers with cobalamin deficiency and the possible role of succinyl-CoA and glycine in the pathogenesis. Medicine (Baltimore) 94(9), e584 (2015).