• Title/Summary/Keyword: PAL enzyme

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Phenylketonuria: Current Treatments and Future Developments (페닐케톤뇨증의 치료: 현재와 미래)

  • Lee, Jeongho
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.20 no.2
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    • pp.37-43
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    • 2020
  • Phenylketonuria is the most prevalent disorder caused by an inborn error in aminoacid metabolism. It results from mutations in the phenylalanine hydroxylase (PAH) gene. If untreated or late treated, results in profound and irreversible mental disability. Newborn screening test identify patients with phenylketouria. The early initiation of a phenylalanine restricted diet very soon prevents most of the neuropsychiatric complications. However, the diet therapy is difficult to maintain and compliance is poor, especially in adolescents and adulthood. Since 2015, American Medical College of Medical Genetics and Genomics (ACMG) recommended more strong restrictive diet therapy for target blood level of phenylalanine (<360 umol/L). For over four decades the only treatment was a very restrictive low phenylalanine diet. This changed in 2007 with the approval of cofactor therapy (Tetrahydrobiopterin, BH4) which is effective in up to 30% of patients. Data from controlled clinical trials with sapropterin dihydrochloride indicate a similar occurrence of all-cause adverse events with this treatment and placebo. Large neutral aminoacids (LNAA) competes with phenylalanine for transport across the blood-brain-barrier and have a beneficial effect on executive functioning. A new therapy has just been approved that can be effective in most patients with PAH deficiency regardless of their degree of enzyme deficiency or the severity of their phenotype. Phenylalanine ammonia lyase (PAL-PEG) was approved in the USA by FDA in May of 2018 for adult patients with uncontrolled blood phenylalanine concentrations on current treatment. Nucleic acid therapy (therapeutic mRNA or gene therapy) is likely to provide longer term solutions with few side effects.

Metabolic Elasticity and Induction of Heat Shock Protein 70 in Labeo rohita Acclimated to Three Temperatures

  • Das, T.;Pal, A.K.;Chakraborty, S.K.;Manush, S.M.;Chatterjee, N.;Apte, S.K.
    • Asian-Australasian Journal of Animal Sciences
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    • v.19 no.7
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    • pp.1033-1039
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    • 2006
  • The metabolic response of Labeo rohita to thermal acclimation was assessed. Advanced fingerlings of L. rohita (average weight $31{\pm}1.4g$) were acclimated to 31, 33 and $36^{\circ}C$ compared with ambient temperatures ($26^{\circ}C$) for 30 days and different enzymes associated with stress response were estimated. Glycolytic enzyme-Lactate dehydrogenase, (LDH, E.C.1.1.1.27), TCA cycle enzyme-Malate dehydrogenase (MDH, E.C.1.1.1.37), Protein metabolizing enzymes-Aspartate amino transferase (AST, E.C.2.6.1.1) and Alanine amino transferase (ALT, E.C.2.6.1.2) of liver, gill and muscle, Gluconeogenic enzymes-Fructose 1,6 Bi phosphatase (FBPase, E.C. 3.1.3.11) and Glucose 6 phosphatase (G6Pase, E.C. 3.1.3.9) of liver and kidney were significantly (p<0.05) different with increasing acclimation temperatures. Heat Shock Protein-70 (HSP-70) was expressed in increasing intensity at 31, 33 and $36^{\circ}C$ but was not expressed at $26^{\circ}C$. Results suggest that higher acclimation temperatures enhance metabolism and L. rohita maintains homeostasis between $26-36^{\circ}C$ via an acclimation episode. Such adaptation appears to be facilitated by resorting to gluconeogenic and glycogenolytic pathways for energy mobilization and induction of HSPs.

Detection of Polymorphism of Growth Hormone Gene for the Analysis of Relationship between Allele Type and Growth Traits in Karan Fries Cattle

  • Pal, Aruna;Chakravarty, A.K.;Bhattacharya, T.K.;Joshi, B.K.;Sharma, Arjava
    • Asian-Australasian Journal of Animal Sciences
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    • v.17 no.10
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    • pp.1334-1337
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    • 2004
  • The present study was conducted to detect polymorphism at growth hormone gene in Karan Fries bulls. A 428 bp fragment of growth hormone gene spanning over $4^{th}$exon, $4^{th}$intron and $5^{th}$ exon was amplified and digested with AluI restriction enzyme to identify polymorphism at this locus. Karan Fries bulls were found to be polymorphic at this locus. Two genotypes LL and LV were identified in Karan Fries with higher allelic frequency for L allele. In Karan Fries males, the average birth weight, 3 months body weight and daily body weight gains of LL homozygotes were significantly higher than that of LV heterozygotes. Genetic distances of KF bulls with respect to genotype along with 3 months body weight and average daily body weight gain forms a single cluster of bulls with LL genotype, while individuals with LV genotype forms three distinct clusters indicating more influence of L allele on growth traits.

Gelatinized Carbohydrates in the Diet of Catla catla Fingerlings: Effect of Levels and Sources on Nutrient Utilization, Body Composition and Tissue Enzyme Activities

  • Yengkokpam, Sona;Sahu, N.P.;Pal, A.K.;Mukherjee, S.C.;Debnath, Dipesh
    • Asian-Australasian Journal of Animal Sciences
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    • v.20 no.1
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    • pp.89-99
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    • 2007
  • A Feeding trial was conducted to study the effects of three different sources and two levels of dietary gelatinized carbohydrate (GC) on nutrient utilization, growth, tissue composition and tissue enzyme activities of fingerlings of Catla catla (15.1-15.3 g). Six isocaloric (17.1-17.5 kJ/g) semi-purified diets were prepared either with rice, corn or tapioca at 40 or 50% GC each. The crude protein (CP) level used in the diet was 35% and 25% for 40% and 50% GC level, respectively to study the protein sparing effect of GC. The degree of gelatinization was higher for corn and tapioca than rice under similar cooking conditions. After a 60-d feeding trial, dry matter, carbohydrate, protein and lipid digestibility were higher in tapioca fed groups at both the levels of GC. However, the highest specific growth rate (SGR) and protein efficiency ratio (PER) were observed in the corn fed groups at 50% GC level indicating better utilization of nutrients from gelatinized corn. Feed conversion ratio (FCR) was almost similar in corn and tapioca fed groups between two levels of GC but in rice fed groups, FCR was lower in 40% GC than 50% GC level. The results indicated higher protein-sparing effect in corn and tapioca fed groups than rice fed groups. The order of gelatinized carbohydrate utilization in Catla catla fingerlings at 50% GC level was corn>tapioca>rice. At 40% GC level, corn and tapioca were comparable and more efficiently utilized than rice. In the corn fed groups, 50% GC was comparable with 40% GC level, whereas in rice and tapioca fed groups the 40% GC was better in terms of nutrient utilization. Liver glycogen content and hepatosomatic index were significantly (p<0.05) higher in those groups fed high GC (50%) irrespective of carbohydrate sources. Higher intestinal amylase and glucose-6-phosphate dehydrogenase activities were observed in higher GC fed groups than the lower GC groups. No mortality was found in any groups at any levels of GC.

Effects of Pseudomonas aureofaciens 63-28 on Defense Responses in Soybean Plants Infected by Rhizoctonia solani

  • Jung, Woo-Jin;Park, Ro-Dong;Mabood, Fazli;Souleimanov, Alfred;Smith, Donald L.
    • Journal of Microbiology and Biotechnology
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    • v.21 no.4
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    • pp.379-386
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    • 2011
  • The objective of this work was to investigate the ability of the plant growth-promoting rhizobacterium Pseudomonas aureofaciens 63-28 to induce plant defense systems, including defense-related enzyme levels and expression of defense-related isoenzymes, and isoflavone production, leading to improved resistance to the phytopathogen Rhizoctonia solani AG-4 in soybean seedlings. Seven-day-old soybean seedlings were inoculated with P. aureofaciens 63-28, R. solani AG-4, or P. aureofaciens 63-28 plus R. solani AG-4 (P+R), or not inoculated (control). After 7 days of incubation, roots treated with R. solani AG-4 had obvious damping-off symptoms, but P+R-treated soybean plants had less disease development, indicating suppression of R. solani AG-4 in soybean seedlings. Superoxide dismutase (SOD) and catalase (CAT) activities of R. solani AG-4-treated roots increased by 24.6% and 54.0%, respectively, compared with control roots. Ascorbate peroxidase (APX) and phenylalanine ammonia lyase (PAL) activities of R. solani AG-4-treated roots were increased by 75.1% and 23.6%, respectively. Polyphenol oxidase (PPO) activity in soybean roots challenged with P. aureofaciens 63-28 and P+R increased by 25.0% and 11.6%, respectively. Mn-SOD (S1 band on gel) and Fe-SOD (S2) were strongly induced in P+R-treated roots, whereas one CAT (C1) and one APX (A3) were strongly induced in R. solani AG-4- treated roots. The total isoflavone concentration in P+Rtreated shoots was 27.2% greater than the control treatment. The isoflavone yield of R. solani AG-4-treated shoots was 60.9% less than the control.

Bibliographical Study on Microorganims of Traditional Korean Nuruk(Since 1945) (한국 전통 누룩 미생물의 문헌적 고찰(1945년 이후를 중심으로))

  • Yu, Tae-Shick;Kim, Jung;Kim, Hyun-Soo;Hyun, Ji-Suk;Ha, Hyun-Pal;Park, Moon-Geun
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.27 no.4
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    • pp.789-799
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    • 1998
  • Literatures on microorganisms of traditional Korean nuruk published since 1945 were reviewed in this paper. Traditional Korean nuruk consists of raw barley and various grains. Traditional Korean nuruk consists of unbolied raw barely and various grains. They are ground to paste and moistened, and then naturally inoculated by airborne microorganisms. Therefore, many kinds of microorganisms such as fungi, yeast, and bacteria grwo in nuruk. Since 1945, new 14 species of Aspergillus and 9 species of Penicillium have been identified from traditional Korean nuruk. Total number of fungal species identified so far is now up to 38 species among 12 different genus. Among newly isolated fungal species, Aspergillus penicilloides and Penicillium, expansum showed not only high production rate of acid and amylase but also extreme stability of the enzyme at room temperature for 3 months. As examples of newly isolated yeast species, there are 5 species of Candida, 4 species of Hansenula, 1 species of Pichia and 1 species of Schizosaccharomyces. Total number of yeast species isolated so far is up to 18 species from different 8 genus. Newly isolated bacteria, were Bacillus pumilus, Lactobacillus casei and Leuconostoc mesenteroides.

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Regional Difference of ROS Generation, lipid Peroxidation, and Antioxidant Enzyme Activity In Rat Brain and Their Dietary Modulation

  • Baek, Bong-Sook;Kwon, Hyun-Joo;Lee, Kyoung-Hee;Yoo, Mi-Ae;Kim, Kyu-Won;Yuji-Ikeno;Yu, Byung-Pal;Chung, Hae-Young
    • Archives of Pharmacal Research
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    • v.22 no.4
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    • pp.361-366
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    • 1999
  • One of the potential causes of age-related neuronal damage can be reactive oxygen species (ROS), as the brain is particularly sensitive to oxidative damage. In the present study, we investigated the effects of aging and dietary restriction (DR) on ROS generation, lipid peroxidation, and antioxidant enzymes in cerebrum, hippocampus, and cerebellum of 6-, 12-, 18-, and 24-month-old rats. ROS generation significantly increased with age in cerebrum of ad libitum (AL) rats. However, no significant age-difference was observed in hippocampus and cerebellum. DR significantly decreased ROS generation in cerebrum and cerebellum at 24-months. On the other hand, the increased lipid peroxidation of AL rats during aging was significantly reduced by DR in all regions. Our results further showed that catalase activity decreased with age in cerebellum of AL rats, which was reversed by DR, although SOD activity had little change by aging and DR in all regions. In a similar way, glutathione (GSH) peroxidase activity increased with age in cerebrum of AL rats, while DR suppressed it at 24-months. These data further support the evidence that the vulnerability to oxidative stress in the brain is region-specific.

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Antifungal Activities of Crude Extractum from Camellia semiserrata Chi (Nanshancha) Seed Cake Against Colletotrichum musae, Colletotrichum gloeosporioides and Penicillium italicum in vitro and in vivo Fruit Test

  • Meng, Xiangchun;Li, Jun;Bi, Fangcheng;Zhu, Lixue;Ma, Zhiyu
    • The Plant Pathology Journal
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    • v.31 no.4
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    • pp.414-420
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    • 2015
  • Antifungal activities of crude extractum of Nanshancha Seed Cake (NSC), to inactivate postharvest pathogens were investigated. Highest inhibitory rate was found against C. musae, C. gloeosporioides and C. papaya P.Henn, which was much stronger than that by tea saponin. Compared to tea saponin, effects of NSC extractum was relatively weak and similar on C. gloeosporioides Penzig and P. italicum. In an in vivo study, best controlling effects by NSC extractum was found with banana anthracnose disease development, which showed no inhibitory effects by tea saponin. NSC extractum controlled in vitro C. musae growth through directly inhibiting germination rate and germ tube elongation, and causing distortation, rupture and indentation of C. musae mycelium. In banana fruit subject to C. musae inoculation, higher PAL, POD, GLU and CHT activity was observed in banana fruit treated with crude NSC extractum than that of water control fruits. Current study proved the best controlling effects of crude NSC extractum in C. musae in vitro and in vivo development, which through direct inhibition of C. musae growth and increasing defense system of the banana fruit.

Inhibitory Effect of Chitosan and Phosphate Cross-linked Chitosan against Cucumber Mosaic Virus and Pepper Mild Mottle Virus

  • Gangireddygari, Venkata Subba Reddy;Chung, Bong Nam;Cho, In-Sook;Yoon, Ju-Yeon
    • The Plant Pathology Journal
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    • v.37 no.6
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    • pp.632-640
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    • 2021
  • Cucumber mosaic virus (CMV) and Pepper mild mottle virus (PMMoV) causes severe economic loss in crop productivity of both agriculture and horticulture crops in Korea. The previous surveys showed that naturally available biopolymer material - chitosan (CS), which is from shrimp cells, reduced CMV accumulation on pepper. To improve the antiviral activity of CS, it was synthesized to form phosphate cross-linked chitosan (PCS) and compared with the original CS. Initially, the activity of CS and PCS (0.01%, 0.05%, and 0.1% concentration) compound against PMMoV infection and replication was tested using a half-leaf assay on Nicotiana glutinosa leaves. The total number of local lesions represented on a leaf of N. glutinosa were counted and analyzed with phosphate buffer treated leaves as a negative control. The leaves treated with a 0.1% concentration of CS or PCS compounds exhibited an inhibition effect by 40-75% compared with the control leaves. The same treatment significantly reduced about 40% CMV accumulation measured by double antibody sandwich enzyme-linked immunosorbent assay and increased the relative expression levels of the NPR1, PR-1, cysteine protease inhibitor gene, LOX, PAL, SRC2, CRF3 and ERF4 genes analyzed by quantitative reverse transcriptase-polymerase chain reaction, in chili pepper plants.

Biocontrol of Damping-Off(Rhizoctonia solani) in Cucumber by Trichoderma asperellum T-5 (Trichoderma asperellum T-5를 이용한 오이 모잘록병(Rhizoctonia solani)의 생물학적 제어)

  • Ryu, Ji-Yeon;Jin, Rong-De;Kim, Yong-Woong;Lee, Hyang-Burm;Kim, Kil-Yong
    • Korean Journal of Soil Science and Fertilizer
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    • v.39 no.4
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    • pp.185-194
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    • 2006
  • A fungal strain of Trichoderma having strong chitinolytic activity was isolated from field soil enriched with crabshell for several years. Based on 5.8S rRNA, partial 18S, 28S rRNA genes, ITS1, ITS2 sequence analysis and morphological characteristics, the fungus was identified as Trichoderma asperellum and named as Trichoderma asperellum T-5 (TaT-5). The fungus released lytic enzymes such as chitinase and ${\beta}$-1, 3-glucanse, and produced six antifungal substances in chitin broth medium. To demonstrate the protective effect of TaT-5 against damping-off in cucumber plant caused by Rhizoctonia solani, TaT-5 culture broth (TA), chitin medium (CM) and distilled water (DW) were applied to each pot at 10 days after sowing, respectively. Then, the homogenized hyphae of R. solani were infected to each pot at 1 week after TaT-5 inoculation. During experimental period, fresh weight of shoot and root in cucumber plant more increased at TA treatment compared to other treatments. PR-proteins (${\beta}$-1, 3-glucanase and chitinase) activities in cucumber leaves markedly increased at CM and DW treatments, but the activity slightly increased and then decreased at TA treatment at 3 days after infection of R. solani. The activity of PR-proteins activities in cucumber roots at all treatments decreased with time where the degree of decrement was more alleviated at TA treatment than CM and DW. These results suggest that the lytic enzymes (chitinase and ${\beta}$-1, 3-glucanse) and antifungal substances produced by TaT-5 can reduce the pathogenic attack by R. solani in cucumber plants.