• Journal of the Korean Society of Food Science and Nutrition
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• v.32 no.6
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• pp.806-811
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• 2003

Effects of pH, temperature, light, sugars, organic acids, metal ions, ascorbic acid, thiamine nicotinic acid and pyridoxine on the stability of the color of the cranberry solution were investigated. The pH had marked influences on the color of the cranberry solution: i.e., the color of the cranberry solution was more intense at low pH. It showed characteristic bathochromic shift as the pH of the solution increased. The half-lives of olor value were 34 days at 37$^{\circ}C$, 91 mins at 9$0^{\circ}C$ and 29 mins at 12$0^{\circ}C$. Light gave an adverse effect to the stability of the color. The color degradation can be minimized by shielding the light from the cranberry solution. Among the sugars tested, fructose was the most deleterious followed by sucrose, galactose, maltose and glucose. Fumaric acid was found to be the most effective in stabilizing the color followed by citric acid, malic acid, acetic acid, while tartaric acid was found to be deleterious. Among the metal ions tested N $a^{+}$ and $Mg^{2+}$ were found to be effective in stabilizing the color, while M $n^{2+}$ was found to be the most deleterious followed by F $e^{2+}$, $K^{+}$ and $Ca^{2+}$. Ascorbic acid was found to be deleterious considerably followed by thiamine, while nicotinic acid and pyridoxine were found to be effective in stabilizing the color feebly.or feebly.

• BMB Reports
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• v.41 no.5
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• pp.408-413
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• 2008

Pyridoxal-5'-phosphate phosphatase (PLPP) catalyzes the dephosphorylation of pyridoxal-5'-phosphate (PLP). A human brain PLPP gene was fused with a PEP-1 peptide and produced a genetic in-frame PEP-1-PLPP fusion protein. The purified PEP-1-PLPP fusion protein was efficiently transduced into PC12 cells in a time- and dose-dependent manner when added exogenously to culture media. Once inside the cells, the transduced PEP-1-PLPP fusion protein was stable for 36 h. The concentration of PLP was markedly decreased by the addition of exogenous PEP-1-PLPP to media pretreated with the vitamin $B_6$ precursors; pyridoxine, pyridoxal kinase and pyridoxine-5'-phosphate oxidase into cells. The results suggest that the transduction of the PEP-1-PLPP fusion protein can be one mode of PLP level regulation, and to replenish this enzyme in the various neurological disorders related to vitamin $B_6$.

• Biotechnology and Bioprocess Engineering:BBE
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• v.4 no.1
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• pp.21-25
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• 1999

Two low-salt complex media, bactopeptone and desalted yeast extract, were used for high density cultivation of the hyperthermophilic archaeon Sulfolobus solfataricus (DSM 1617). Bactopeptone, which has low mineral ion content among various complex media, was good for cell growth in batch cultures; the maximal cell density in bactopeptone was comparable to that in yeast extract. However, cell growth was rather poor when bactopeptone was added by the fed-batch procedure. Since several vitamins are deficient in abctopeptone, the effect of vitamins on cell growth was examined. Among the vitamins tested, pyridoxine was found to improve the growth rate of S. solfataricus. To reduce the growth inhibition caused by mineral ions, yeast extract was dialyzed against distilled water and then fed-batch cultures were carried out using a fed medium containing desalted yeast extract. Although the concentrations of mineral ions in yeast extract were significantly lowered by the dialysis whether low molecular weight solutes in yest extract are crucial for cell growth, we investigated the effect of trehalose, a most abundant compatible solute in yeast extract, on the growth pattern. Cell densities were increased and the length of the lag phase was markedly shortened by the presence of trehalose, indicating that trehalose plays an important role in the growth of S. solfataricus.

• Asian Pacific Journal of Cancer Prevention
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• v.13 no.11
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• pp.5333-5338
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• 2012

Vitamin B6 functions as a coenzyme in >140 enzymatic reactions involved in the metabolism of amino acids, carbohydrates, neurotransmitters, and lipids. It comprises a group of three related 3-hydroxy-2-methyl-pyrimidine derivatives: pyridoxine (PN), pyridoxal (PL), pyridoxamine (PM) and their phosphorylated derivatives [pyridoxal 5'-phosphate (PLP) and pyridoxamine 5'-phosphate (PMP)], In the folate metabolism pathway, PLP is a cofactor for the mitochondrial and cytoplasmic isozymes of serine hydroxymethyltransferase (SHMT2 and SHMT1), the P-protein of the glycine cleavage system, cystathionine ${\beta}$-synthase (CBS) and ${\gamma}$-cystathionase, and betaine hydroxymethyltransferase (BHMT), all of which contribute to homocysteine metabolism either through folate-mediated one-carbon metabolism or the transsulfuration pathway. Folate cofactors carry and chemically activate single carbons for the synthesis of purines, thymidylate and methionine. So the evidence indicates that vitamin B6 plays an important role in maintenance of the genome, epigenetic stability and homocysteine metabolism. This article focuses on studies of strand breaks, micronuclei, or chromosomal aberrations regarding protective effects of vitamin B6, and probes whether it is folate-mediated one-carbon metabolism or the transsulfuration pathway for vitamin B6 which plays critical roles in prevention of cancer and cardiovascular disease.

• BMB Reports
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• v.30 no.2
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• pp.125-131
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• 1997

This work reports studies of the catalytic and structural properties of pyridoxal kinase (ATP: pyridoxal 5' -phosphotransferase, EC. 2.7.1.35), Pyridoxal kinase catalyzes the phosphorylation of vitamin $B_6$ (pyridoxal, pyridoxamine, pyridoxine) using ATP-Zn as a phosphoryl donor. The enzyme purified from brain tissues is made up of two identical subunits of 40 kDa each. Native enzyme was inhibited by a substrate analogue, pyridoxal-oxime. Limited chymotrypsin digestion of pyridoxal kinase yields two fragments of 24 and 16 kDa with concomitant loss of catalytic activity. These fragments were isolated by DEAE ion exchange chromatography and used for binding studies with fluorescent ATP and pyridoxal analogues. The spectroscopic properties of both fluorescent pyridoxal analogue and Anthraniloyl ATP (Ant-ATP) bound to the 24 kDa fragment are indistinguishable from those of both pyridoxal analogue and Ant-ATP bound to the native pyridoxal kinase, respectively. The small 16 kDa fragment, generated by proteolytic cleavage of the kinase, does not bind any of the substrate analogues. Binding characteristics of Ant-ATP were extensively studied by measuring the changes in fluorescence spectra at various conditions. From the results presented herein, it is postulated that the structural domain associated with catalytic activity comprises approximately one-half of the molecular mass of pyridoxal kinase (24 kDa). whereas the remaining portion (16 kDa) of the enzyme contains a regulatory binding domain.

• Journal of Pharmaceutical Investigation
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• v.9 no.3
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• pp.1-8
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• 1979

The main route of metabolism of isonicotinic acid hydrazid (INH) in man is its conjugation with acetyl coenzyme A to form acetyl-INH. The reaction is catalyzed by an N-acetyl transferase in the liver. The acetylated drug can be excreted by the kidney more efficiently than INH, and the biological half-life of the drug in the body depends upon how rapidly the drug can be acetylated. This report measured the concentration of INH in the blood of 147 individuals 6 hours after they received a standard dose (9.8mg/kg) and plotted the data as a frequeney distribution hiotogram. There was bimodality, with a mean for one subpopulation at approximately $0.6{\sim}0.8\;mcg/ml.$, and a mean for the other subpopulation between 2.8 and 4.0mcg/ml. As might be expected slow acetylators of INH are more likely to develop a cumulative toxicity to the drug. The principle ,toxicity to INH is a peripheral neuritis but this adverse effect can be prevented by given extra pyridoxin to the patients, and the vitamin does not alter the antitubercular activity of INH. This report carried out that pyridoxine does not alter the ratio of free INH to the total INH in blood.

• Natural Product Sciences
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• v.12 no.3
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• pp.166-173
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• 2006

Silymarin is an antihepatotoxic substance isolated from the fruits of silybum mariamum. Possibly due to their antioxidant and membrane stabilizing properties, the compounds was shown to protect various organs and cells against a number of insults (Kvasnicka et al., 2003). Among the main silymarin components, [silybin($SB_A,\;SB_B$, isosilybin ($ISB_A,\;ISB_B$) silydianin (SD) and silychristin (SC)], silybin is the major pharmacologically active compound. Korean Pharmaceutical Codex (2nd ed.) describes silybin as the main substance of Cardus Marianus extract as supportive treatment of chronic inflammatory liver disorders. The aim of this work was to analyze silybin from various preparations containing cardus marianus extract, nicotinamide, and riboflavin (CNR). Nine commercial products were tested using reversed-phase HPLC-PDA assay. The limits of detection and quantification were $0.2\;{mu}g/ml$ and $1\;{mu}g/ml$, respectively. Calibration curve showed a good linearity ($r^2$=1.00000) in the range of $1{\sim}500\;{\mu}g/ml$ of silybin standard solutions.

• Journal of Nutrition and Health
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• v.28 no.4
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• pp.321-330
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• 1995

Concentrations of total vitamin B-6 in human milk as well as individual, B-6 vitamers have important implications for the nutritional management of breast-fed(BF) infants. Vitamin B-6 status was assessed in 3 groups of infants : two groups preterm (PT) BF infants whose mothers were supplemented with 2 or 27mg pyridoxine(PN)-HCI ; a sub group of formula-fed (FF) PT infants. Mothers and infants were assessed weekly during the 28-day post feeding. Throughout the neonatal period, levels of total vitamin B-6 and percentages of pyridoxal(PL) in breast milk were lower in PT than T mothers, even in mothers supplemented with 27mg PN-HCI. Total vitamin B-6 levels in PT milk paralleled maternal supplementation but percentage distributions of B-6 vitamers did not change. Vitamin B-6 intakes of BF preterm infants paralleled their mothers' level of infants in the 2mg group was suggested by vitamin status parameters. Vitamin B-6 inadequacy of infants correlated with their plasma pyridoxal-5-phosphate(PLP) levels and erythrocyte alanine aminotransferase(E-ALAT) activity; all parameters such as plasma PLP, PL/PLP ratio and stimulation % of E-ALAT were highest for FF PT infants. The positive correlation of vitamin B-6 levels in breast milk gestational age may contraindicate its adequacy for some PT infants.

• Clinical and Experimental Pediatrics
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• v.58 no.11
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• pp.407-414
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• 2015

Early-onset epileptic encephalopathies are one of the most severe early onset epilepsies that can lead to progressive psychomotor impairment. These syndromes result from identifiable primary causes, such as structural, neurodegenerative, metabolic, or genetic defects, and an increasing number of novel genetic causes continue to be uncovered. A typical diagnostic approach includes documentation of anamnesis, determination of seizure semiology, electroencephalography, and neuroimaging. If primary biochemical investigations exclude precipitating conditions, a trial with the administration of a vitaminic compound (pyridoxine, pyridoxal-5-phosphate, or folinic acid) can then be initiated regardless of presumptive seizure causes. Patients with unclear etiologies should be considered for a further workup, which should include an evaluation for inherited metabolic defects and genetic analyses. Targeted next-generation sequencing panels showed a high diagnostic yield in patients with epileptic encephalopathy. Mutations associated with the emergence of epileptic encephalopathies can be identified in a targeted fashion by sequencing the most likely candidate genes. Next-generation sequencing technologies offer hope to a large number of patients with cryptogenic encephalopathies and will eventually lead to new therapeutic strategies and more favorable long-term outcomes.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.9-17
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• 2005

Homocystinuria is a metabolic disorder caused by a deficiency of cystathionine ${\beta}$-synthase (CBS). Patients with homocystinuria show clinical symptoms such as mental retardation, lens dislocation, vascular disease with life-threatening thromboembolisms and skeletal deformities. Generally, the major treatments for CBS deficiency include pharmacologic doses of pyridoxine or dietary restriction of methionine. However, there is no effective treatment for this disease up till today and gene therapy can be an attractive novel approach to treatment of the disease. We investigated whether a recombinant adeno-associated virus could be used as a CBS gene transfer vector to reduce the excessive homocysteine level in the homocystinuria mouse model. Recombinant adeno-associated virus vector encoding the human CBS gene (rAAV-hCBS), driven by EF1-a promoter, was infused into CBS-deficient mice ($CBS^{-/-}$) via intramuscular (IM) and intraperitoneal (IP) injection. IP injection was more efficient than IM injection for prolongation of lives and reduction of plasma homocysteine levels. After 2 weeks of gene transfer by IP injection, serum homocysteine level was significantly decreased in treated mice compared with the age-matched controls and the life span was extended about 1.5 times. Also, increased expression of CBS gene was observed by immunohistochemical staining in livers of treated $CBS^{-/-}$ mice and microvesicular lipid droplets was decreased in cytoplasm of liver. These results demonstrate the possibility and efficacy of gene therapy by AAV gene transfer in homocystinuria mice.