• Molecules and Cells
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• v.38 no.3
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• pp.259-266
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• 2015

The regulation of flowering time has crucial implications for plant fitness. MicroRNA156 (miR156) represses the floral transition in Arabidopsis thaliana, but the mechanisms regulating its transcription remain unclear. Here, we show that two AGAMOUS-like proteins, AGL15 and AGL18, act as positive regulators of the expression of MIR156. Small RNA northern blot analysis revealed a significant decrease in the levels of mature miR156 in agl15 agl18 double mutants, but not in the single mutants, suggesting that AGL15 and AGL18 co-regulate miR156 expression. Histochemical analysis further indicated that the double mutants showed a reduction in MIR156 promoter strength. The double mutants also showed reduced abundance of pri-miR156a and pri-miR156c, two of the primary transcripts from MIR156 genes. Electrophoretic mobility shift assays demonstrated that AGL15 directly associated with the CArG motifs in the MIR156a/c promoters. AGL18 did not show binding affinity to the CArG motifs, but pull-down and yeast two-hybrid assays showed that AGL18 forms a heterodimer with AGL15. GFP reporter assays and bimolecular fluorescence complementation (BiFC) showed that AGL15 and AGL18 co-localize in the nucleus and confirmed their in vivo interaction. Overexpression of miR156 did not affect the levels of AGL15 and AGL18 transcripts. Taking these data together, we present a model for the transcriptional regulation of MIR156. In this model, AGL15 and AGL18 may form a complex along with other proteins, and bind to the CArG motifs of the promoters of MIR156 to activate the MIR156 expression.

• Korean journal of food and cookery science
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• v.22 no.5 s.95
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• pp.728-735
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• 2006

This study investigated the development of health promoting high quality Mumalangi Kimchi. Angelica gigas Nakai leaves (AGL) were extracted with water and 70% ethanol, and the extracts tested for their electron donating ability (EDA), nitrite scavenging ability (NSA) and inhibitory effects on MDA and A549 cells. The EDA in 100-1,000 ppm water extracts from AGL ranged from 40 to 80%, but that of the ethanol extracts ranged from 37 to 81%. The NSA increased with increasing AGLconcentration in the extracts and decreasing pH. The NSA of the 1,000 ppm water and ethanol extracts from AGL were 29 and 35%, respectively, at pH 1.2. The inhibition ratios of the water and ethanol extracts from AGL on MDA cell growth were 35 and 32%, while those on A549 cell growth were 27 and 23%, respectively, at 1,000 ppm. After sun drying radishes for 15 hours, for the preparation of Mumalangi, the water contents were higher in summer radishes (39.5%) than fall radishes (32.6%) the color of summer radish also changed to brown. During storage of Mumalangi Kimchi, with the addition of 1-3% AGL, at 20?for 4 weeks, the yeast growth was inhibited. The shelf-life of Mumalangi Kimchi was extended by the addition of AGL. In the sensory evaluation of Mumalangi Kimchi, that with the addition 2% AGL had the highest scores for color, flavor, taste, texture, after taste and overall acceptability. Mumalangi Kimchi with the addition of 2% AGL had significant high scores for both taste and overall acceptability (p.0.05).

• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.2
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• pp.108-112
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• 2012

Glycogen storage disease type III (GSD type III, OMIM #232400) is a rare autosomal recessive disease caused by a deficiency of the glycogen-debranching enzyme (GDE) with a mutation in the AGL gene (OMIM *610860). It is known to be bifunctional enzyme, that is, having two independent catalytic activities; 1,4-${\alpha}$-D-glucan 4-${\alpha}$-D-glycosyltransferase (EC 2.4.1.25) and amylo-1,6-glucosidase (EC 3.2.1.33) that occur at separate active sites on a single polypeptide chain. Most patients with GSD type III usually have symptoms related to decreased glycogenolysis in liver and muscles, such as hepatomegaly, hypoglycemia, failure to thrive, hyperlipidemia, muscle weakness and cardiomyopathy (type IIIa), however some patients show symptoms restricted to liver (type IIIb). GSD type III is diagnosed by enzyme test through liver or muscle biopsy or mutation analysis of the AGL gene. We report the case of GSD type III proven by gene study after liver biopsy, which revealed c.476delA, c.3444_3445insA in exon 6, 27 of AGL gene in Korean patient.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• KSII Transactions on Internet and Information Systems (TIIS)
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• v.5 no.11
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• pp.1959-1975
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• 2011

Adaptive Bit Loading (ABL) in Multiple-Input Multiple-Output Orthogonal Frequency-Division Multiplexing (MIMO-OFDM) is often used to achieve the desired Bit Error Rate (BER) performance in wireless systems. In this paper, we discuss some of the bit loading algorithms, compare them in terms of the BER performance, and present an effective and concise Adaptive Grouped Loading (AGL) algorithm. Furthermore, we propose a "weight factor" for loading algorithm to converge rapidly to the final solution for various data rate with variable Signal to Noise Ratio (SNR) gaps. In particular, we consider the bit loading in near optimal Singular Value Decomposition (SVD) based MIMO-OFDM system. While using SVD based system, the system requires perfect Channel State Information (CSI) of channel transfer function at the transmitter. This scenario of SVD based system is taken as an ideal case for the comparison of loading algorithms and to show the actual enhancement achievable by our AGL algorithm. Irrespective of the CSI requirement imposed by the mode of the system itself, ABL demands high level of feedback. Grouped Loading (GL) would reduce the feedback requirement depending upon the group size. However, this also leads to considerable degradation in BER performance. In our AGL algorithm, groups are formed with a number of consecutive sub-channels belonging to the same transmit antenna, with individual gains satisfying predefined criteria. Simulation results show that the proposed "weight factor" leads a loading algorithm to rapid convergence for various data rates with variable SNR gap values and AGL requires much lesser CSI compared to GL for the same BER performance.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.1
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• pp.24-28
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• 2020

Type III Glycogen storage disease (Type III GSD, OMIM#232400) is a genetic metabolic disorder in which undigested glycogen accumulates in the organs due to lack of glycogen debranching enzyme caused by AGL mutation. The clinical symptoms of type III GSD include hepatomegaly, delayed growth, hypoglycemia and muscle weakness. These clinical symptoms are similar to those of other types of GSD, making it difficult to distinguish clinically. The authors report a case of type III GSD diagnosed by gene panel sequencing. A 11-month old male patient was presented with hepatomegaly. In liver biopsy, glycogen was accumulated in hepatocytes, suggesting GSDs. For differential diagnosis of types of GSD, gene panel sequencing for GSDs was performed. As a result, two novel pathogenic compound heterozygous variants: c.311_312del (p.His104Argfs*15) and c.3314+1G>A in AGL were detected and the patient was diagnosed as type III GSD. After diagnosis, he started dietary treatment with cornstarch, and has been free from complications. After two years, two same variants were also identified in the chorionic villous sampling of the pregnant mother, and the fetus was diagnosed as type III GSD. Gene panel sequencing is useful for diagnosis of disease which is indistinguishable by clinically and has high genetic heterogeneity, such as GSD. After diagnosis, familial genetic analysis can provide adequate genetic counseling and rapid diagnosis.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.6 no.1
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• pp.15-23
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• 2006

Purpose: Glycogen storage disease type III (GSD-III), is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme is amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), which is responsible for the debranching of the glycogen molecule during catabolism. The disease has been demonstrated to show clinical and biochemical heterogeneity, reflecting the genotype-phenotype heterogeneity among different patients. In this study, we analyzed mutations of the AGL gene in three unrelated Korean GSD-III patients and discussed their clinical and laboratory implications. Methods: We studied three GSD-III patients and the clinical features were characterized. Sequence analysis of 35exons and part exon-intron boundaries of the AGLgene in patients were carried out by direct DNA sequencing method using genomic DNA isolated from patients' peripheral leukocytes. Results: The clinical features included hepatomegaly (in all patients), seizures (in patient 2), growth failure (in patients 1), hyperlipidemia (in patients 1 and 3), raised transaminases and creatinine kinase concentrations (in all patients) and mild EKG abnormalities (in patients 2). Liver transplantation was performed in patient 2due to progressive hepatic fibrosis. Administration of raw-corn-starch could maintain normoglycemia and improve the condition. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 with c.1510_1511insT (p.Y504LfsX10), and patient 3 with c.3416 T>C (p.L1139P) and c.l735+1 G>T (Y538_R578delfsX4) mutations. Except R428K mutation, 4 other mutations identified in3 patients were novel. Conclusion: GSD-III patients have variable phenotypic characteristics resembling GSD-Ia. The molecular defects in the AGL gene of Korean GSD-III patients were genetically heterogeneous.

• Proceedings of the Korean Society of Potoscience Conference
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• spring
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• pp.34-34
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• 2000

• Proceedings of the Botanical Society of Korea Conference
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• 2000.04a
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• pp.5-5
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• 2000

• Proceedings of the Korean Society of Crop Science Conference
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• 1997.05a
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• pp.40-41
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• 1997