• Journal of Genetic Medicine
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• v.16 no.1
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• pp.19-22
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• 2019

The infantile convulsions and choreoathetosis (ICCA) syndrome is defined when two overlapping clinical features of benign familial infantile epilepsy (BFIE) and paroxysmal kinesigenic dyskinesia (PKD) are present in an individual or a family. Since the gene encoding proline-rich transmembrane protein 2 (PRRT2) was first identified in Han Chinese families with PKD, mutations of PRRT2 have additionally been reported in patients with BFIE and ICCA. We attempted to identify the genetic etiology in an ICCA family where the proband, her elder sister, and a maternal male cousin had BFIE, and her mother had PKD. Whole-exome sequencing performed in the proband and her sister and mother identified a novel pathogenic mutation of PRRT2 (c.640delinsCC; p.Ala214ProfsTer11), which was verified by Sanger sequencing. This frameshift PRRT2 mutation located near the genetic hot spot of base 649_650 results in the premature termination of the protein, as do most previously reported mutations in BFIE, ICCA, and PKD.

• Journal of the Korean Society of Food Science and Nutrition
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• v.34 no.6
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• pp.857-861
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• 2005

The chemical components of different parts of Erigeron annuus were examined in order to use as a new functional food material. Nitrogen free extract contents of flowers, leaves, stems and roots were 53.15, 55.79, 36.71 and $42.61\%$, respectively. The contents of crude fiber in the stems and roots were similar, while those in leaves were lower than those in the flowers. Mineral components of Erigeron annuus were rich in Na $(19.55\~33.78\;mg/100\;g),\;K (49.95\~89.80\;mg/100\;g)\;and\;Ca(25.39\~116.40\;mg/100\;g)$. Among the portions of Erigeron annuus Ca contents was slightly higher level in tile loaves than those of flowers, stems and roots. The major free sugars of Erigeron annuus were sucrose $(0.12\~1.37\%),\;glucose\;(0.68\~1.08\%)\;and\;fructose\;(0.56\~1.66\%)$. The contents of total amino acid in Erigeron annuus were 2,509.74 mg/100 g in the flowers,2,630.95 mg/100 g in the leaves, 889.54 mg/100 g in the stems, and 1,201.41 mg/100 g in leaves. Abundant amino acids in the flowers and leaves were glutamic acid, tyrosine and proline. And abundant amino acids of stems were lysine, glutamic acid and aspartic acid, and its root were lysine, glutamic acid and proline, Organic acid components of Erigeron annuus were succinic acid $(4.78\~19.72\;mg/100\;g),\;tartaric\;acid\;(3.90\~6.91\;mg/100\;g)\;and\;citric\;acid\;(1.79\~6.60\;mg/100\;g)$. Nine fatty acids in Erigeron annuus were identified and the major fatty acids were myristic acid $(18.05\~20.18\%),\;oleic\;acid\;(18.50\~32.91\%)\;and\;linoleic\;acid\;(18.02\~29.87\%)$. Total flavonoid and phenol contents were higher in leaf and flower extracts than any other extracts.

• Food Science and Preservation
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• v.17 no.4
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• pp.526-532
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• 2010

Changes in the nutritional components of Daebong-gam (astringent persimmon) fruit were studied during ripening. The pH rose during ripening and total acidity level fell, from pH 5.36 to pH 5.96 and 0.13% to 0.06%, respectively. Total soluble content did not significantly change. Lightness, redness, and yellowness values, as well as ascorbic acid content, decreased during ripening. The levels of moisture, crude protein, and crude fat also decreased, but nitrogen-free content and crude ash level increased. Daebong-gam was rich in K (96.31~239.47 mg/100 g), P (49.10~55.93 mg/100 g), and Na (15.96~18.13 mg/100 g). Fructose and glucose levels were initially high and increased further during ripening. The glucose content was 4.82% in Daebong-gam, 6.73% in Ban-si, and 7.10% Yeon-Si, respectively. Proline, glutamic acid, and aspartic acid were present at high levels in Daebong-gam. The most common fatty acids were palmitic acid and linolenic acid. Succinic acid was the principal organic acid present.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.26 no.2
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• pp.150-158
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• 1993

In order to investigate the content and seasonal variation of the extractive components including taste compounds, free amino acids, nucleotides and related compounds, quanternary ammonium bases, and guanidino compounds of ascidian collected from the south coast of Korea were determined bimonthy from April to October in 1990. The extractive nitrogen was composed of $60{\sim}62\%$ as free amino acids, $12{\sim}16\%$ as betaines, $5{\sim}9\%$ as nucleotides, and others as trimethylamine oxide(TMAO) and total creatinine. The muscle of ascidian was rich in such free amino acids as taurine, proline, glutamic acid, glycine and glycinebetaine. Most of nitrogenous compounds in the extractives showed a marked seasonal variation with a maximum in summer or autumn. AMP content was relatively high among nucleotides. Succinic acid, malic acid, lactic acid and pyroglutaric acid were the major organic acids in ascidian. The results of omission test suggested that the taste of ascidian is attributed to mainly free amino acid, betaines, nucleotides and nonvolatile organic acid in order.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.5 no.1
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• pp.29-38
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• 1972

The protein composition of abalone muscle was estimated with the following result: on a series of samples analyzed, water-soluble protein, $19\~22\%$, salt-soluble protein, $27\~39\%$: alkali-soluble protein, $20\~26\%$ : and stroma $20\~28\%$ : respectively. It was demonstrated by ultracentrifugal analysis that approximately $65\%$ of the salt-soluble protein is accounted for by paramyosin, $30\%$ by actomyosin, and $5\%$ by myosin, respectively. The ultracentrifugally homogenous paramyosin was prepared by BAILEY's ethanol-dried method. It showed a $S^{\circ}\;_{20,\;{\omega}$ of 3.14s, and was completely salted in with KCl beyond $0.35{\mu}$. The intrinsic viscosity at $25^{\circ}C$ was estimated at 3.1. The paramyosin is rich in several amino acids such as arginine, aspartic acid, glutamic acid, etc., and lacking of both proline and tryptophane, in rough accord with other paramyosins reported. The abalone paramyosin did not show ATPase activity over a pH range of 5 to 9,5 even in the presence of Ca++ or Mg++. So was the case with the paramyosin specimen prepared by BAILEY's wet-extraction method.

• Journal of the Korean Society of Food Science and Nutrition
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• v.35 no.6
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• pp.708-712
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• 2006

The chemical properties of yellow and red onion were investigated and analyzed to provide basic data for functional food materialization and processing. The moisture and nitrogen free extract contents of yellow and red onion were 92.80%, 5.13% and 92.47%, 5.59%, respectively. Mineral components of yellow and red onion were rich in K (123.64 and 114.41 mg%), Na (34.09 and 33.57 mg%) and P (27.04 and 20.56 mg%). The major free sugar of yellow onion were glucose (744.2 mg%) and fructose (705.9 mg%). Whereas major free sugar of red onion were sucrose (692.8 mg%) and fructose (517.3 mg%). Glutamic acid, phenylalanine and aspartic acid in yellow and red onion were major amino acids. Abundant free amino acids in yellow onion were hydroxy-L-proline (27.34 mg%), L-serine (27.34 mg%) and L-arginine (26.25 mg%). Abundant free amino acids in red onion were L-glutamic acid (16.35 mg%), ammonium chloride (15.22 mg%) and L-serine (10.93 mg%). Ascorbic acid contents are higher in red onion (28.34 mg%) than in yellow onion (19.20 mg%). Quercetin and total polyphenol of yellow and red onion were 15.24 mg%, 5.70 mg% and 0.319 mg/g and 0.248 mg/g, respectively.

• Korean Journal of Food Science and Technology
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• v.40 no.5
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• pp.586-592
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• 2008

In this study, the nutritional components and functional activities of Sasa borealis leaf tea were evaluated. The proximate compositions were as follows; moisture 5.68%, crude protein 16.38%, crude fat 4.68%, nitrogen free extracts 32.37%, crude fiber 32.36%, and ash 8.53%, respectively. The mineral elements were as follows: K 2,133.83, Ca 1,144.09 and P 543.00 mg%, respectively. The amino acid contents of the Sasa borealis leaf tea were very rich in proline (1,275.26 mg/100 g) and deficient in cystine (71.49 mg/100 g). The major fatty acid components were linoleic acid (50.52%), palmitic acid (18.52%), and oleic acid (14.16%). Finally, based on our sensory evaluations, the $80^{\circ}C$ extracted Sasa borealis leaf tea evidenced the best overall quality. The contents of total phenol and total flavonoids of the 80% methanol and hot water extracts were 15.09, 7.69 mg/g and 12.03, 6.12 mg/g, respectively. The DPPH and $ABTS^+$ radical scavenging activities of the 80% methanol extract from Sasa borealis leaf tea were 86.87% and 83.85% at a concentration of 1.25 mg/mL. The 80% methanol and hot water extracts evidenced reducing power and inhibitory effects against acetylcholinesterase in a dose-dependent manner.

• Korean Journal of Food Science and Technology
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• v.34 no.2
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• pp.200-206
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• 2002

This study was conducted to identify characteristics of kanjang made with barley bran. Characteristics of kanjang made with barley bran were compared to those of kanjang made with soybean. Total nitrogen was maintained more than 0.7% at 90 days after fermentation. In case of free sugars, the contents of arabinose, xylose, fructose, glucose and maltose were more abundant in kanjang made with barley bran than kanjang made with soybean. The contents of propionic acid and butyric acid were rich among volatile organic acids. Nonvolatile organic acid of lactic acid which was known as abundant component in kanjang was not detected in kanjang made with barley bran. In case of free amino acid, glutamic acid was the most abundant in kanjang made with barley bran, followed by proline and phenylalanine. The ratio of glutamic acid content to total amino acid content was $24.8{\sim}28.0%$. Essential amino acid was $26.7{\sim}29.9%$. Total contents of amino acids were $0.7{\sim}1.4%$. Result of sensory evaluation showed that taste of kanjang made with barley bran was good for fermented after 45 days.

• Korean Journal of Food Science and Technology
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• v.27 no.4
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• pp.471-477
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• 1995

Extractive nitrogen, free amino acids, oligopeptides, nucleotides and related compounds, quaternary ammonium bases, and guanidino compounds were analyzed to evaluate quality of anchovy sauce. The commercial products contained low proximate composition, extractive nitrogen and other extractive components, than the experimentally prepared anchovy sauce. Both samples, commercial products and experimentally prepared anchovy sauce, were rich in free amino acids, such as glutamic acid, leucine, alanine, lysine, and aspartic acid. The extractive nitrogenous components which consist of total nucleotides and related compounds, total free amino acids, methionine, isoleucine, valine, taurine, tyrosine, histidine, leucine, aspartic acid, cystine, and lysine, showed significant correlation(p<0.01) with extractive nitrogen. Possibly, seven kinds of free amino acids such as methionine, isoleucine, valine, taurine, tyrosine, histidine, and leucine, might be recommend as quality indices of standardization for anchovy sauce.

• Food Science and Preservation
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• v.18 no.3
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• pp.341-348
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• 2011

The nutritional components and antioxidative activities of jujube fruit and leaf were investigated and analyzed to provide basic data for functional food materialization and processing. The nitrogen-free extract contents of the fruit and leaf were 71.92% and 41.51%, respectively. The mineral components of the fruit and leaf were rich in Ca (72.14 and 3,252.09 mg/100 g), K (899.82 and 1,708.12 mg/100 g), and P (172.11 and 286.28 mg/100 g), respectively. The major free sugars of the fruit were glucose (13.01 %) and fructose (7.35%); and of the leaf, sucrose (3.94%) and fructose (0.75%). The ascorbic acid contents were higher in fruit (135.73 mg/100 g) than in the leaf (100.43 mg/100 g). The analysis of the component amino acid showed a relatively high ratio of glutamic acid, aspartic acid, proline, and essential amino acids of leucine, but a low methionine and cystine content. The ABTS and FRAP assays indicated that the butanol fraction of the leaf was a more potent radical scavenger and reducing agent than the other five solvent fractions. The butanol fraction of the leaf also presented inhibitory effects against lipid peroxidation in a dose-dependent manner. Therefore, this study verified that the butanol fraction of the leaf has strong antioxidative activities that are correlated with its high level of phenolics, particularly rutin and quercitrin. These phenolics of jujube leaf can be utilized as effective and safe functional food substances, i.e., natural antioxidants.