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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 (Item No. 18425) deficiency can disrupt heme and energy production, leading to neuromotor dysfunction.3
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1. ACYL phosphates as enzyme-
2. Porphyrin and heme metabolism and the porphyrias. Compr Physiol 3(1), 365-401 (2013).
3. Growth retardation, general hypotonia, and loss of acquired neuromotor skills in the infants of mothers with cobalamin deficiency and the possible role of succinyl-