• Journal of the Korean Society of Food Culture
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• v.36 no.5
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• pp.502-511
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• 2021

In this study, the content of water-soluble vitamins such as vitamin B₁ (thiamin), B₂ (riboflavin), B₃ (niacin), B₅ (pantothenic acid), B₆ (pyridoxine), B₇ (biotin), B₁₂ (cyanocobalamin), and C (ascorbic acid) in dishes containing meat and seafood consumed in Korea were determined by high-performance liquid chromatography (HPLC) with ultraviolet and fluorescence detection. All analyses were performed under strict quality control of vitamins B₁, B₂, B₃, B₅, B₆, B₇, B₁₂, and C. The highest content of vitamin B₁ was observed in Bugeo-gangjeong (1.373 mg/100 g) and the highest level of vitamin B₂ (5.162 mg/100 g) was found in pig liver. Bugeo-gangjeong showed the highest content of vitamin B₃ (21.676 mg/100 g), and kkomak-muchim contained considerable amounts (43.310 mg/100 g) of vitamin B₅. Vitamin B₆ was not detected in most seafood dishes except for yangnyeom myeongran-jeot (0.274 mg/100 g) and was present at low levels or not present at all in meat dishes. The highest content of vitamin B₇ was 6.506 ㎍/100 g in saeu-jeon and kkomak-muchim showed the highest content (21.132 ㎍/100 g) of vitamin B₁₂. The highest content of vitamin C was in yangnyeom myeongran-jeot (84.508 mg/100 g). In addition, the analysis methods of each water-soluble vitamin were verified. These results showed that seafood-based ingredients in several dishes could be a good source of water-soluble vitamins.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.19 no.1
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• pp.12-19
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• 2019

Purpose: Isolated methylmalonic acidemia (MMA) is an autosomal recessive inherited disorder of propionate metabolism. There are two subtypes of MMUT gene defects. $Mut^0$ represents complete loss of methylmalonyl-CoA mutase (MCM) activity while mut- is associated with residual MCM activity, which can be stimulated by hydroxocobalamin (OHCbl) supplementation. The objective of this study is to investigate cobalamin responsiveness and mutations present in Korean MMA population. Methods: We evaluated 10 MMA patients using somatic cell complementation analysis on their fibroblasts to measure MCM activity and vitamin B12 responsiveness for the optimal treatment. MMUT gene was sequenced to identify the MMA mutations. Results: For all patients, the incorporation of $[^{14}C]-propionate$ was low, and there was no response to OHCbl. The incorporation of $[^{14}C]-methyltetrahydrofolate$ and $[^{57}Co]-CNCbl$ fell within the normal range. There was adequate synthesis of methylcobalamin while the synthesis of adenosylcobalamin was low. The complementation analysis showed all patients were $mut^0$. The sequence analysis identified 12 different MMUT mutations, including 2 novel mutations, p.Gln267Ter and p.Ile697Phe, were identified. All the patients in this study had neonatal onset of symptoms, belonged to $mut^0$ complementation class, and as a result, showed no cobalamin responsiveness. Conclusion: No Korean MMA patient showed cobalamin responsiveness.