• Title/Summary/Keyword: Leucine

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Long-term Clinical Consequences in Patients with Urea Cycle Disorders in Korea: A Single-center Experience (요소회로대사 질환 환자들의 장기적인 임상 경과에 대한 단일 기관 경험)

  • Lee, Jun;Kim, Min-ji;Yoo, Sukdong;Yoon, Ju Young;Kim, Yoo-Mi;Cheon, Chong Kun
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.21 no.1
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    • pp.15-21
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    • 2021
  • Purpose: Urea cycle disorder (UCD) is an inherited inborn error of metabolism, acting on each step of urea cycle that cause various phenotypes. The purpose of the study was to investigate the long-term clinical consequences in different groups of UCD to characterize it. Methods: Twenty-two patients with UCD genetically confirmed were enrolled at Pusan National University Children's hospital and reviewed clinical features, biochemical and genetic features retrospectively. Results: UCD diagnosed in the present study included ornithine transcarbamylase deficiency (OTCD) (n=10, 45.5%), argininosuccinate synthase 1 deficiency (ASSD) (n=6, 27.3%), carbamoyl-phosphate synthetase 1 deficiency (CPS1D) (n=3, 13.6%), hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHHS) (n=2, 9.1%), and arginase-1 deficiency (ARG1D) (n=1, 4.5%). The age at the diagnosis was 32.7±66.2 months old (range 0.1 to 228.0 months). Eight (36.4%) patients with UCD displayed short stature. Neurologic sequelae were observed in eleven (50%) patients with UCD. Molecular analysis identified 37 different mutation types (14 missense, 6 nonsense, 6 deletion, 6 splicing, 3 delins, 1 insertion, and 1 duplication) including 14 novel variants. Progressive growth impairment and poor neurological outcomes were associated with plasma isoleucine and leucine concentrations, respectively. Conclusion: Although combinations of treatments such as nutritional restriction of proteins and use of alternative pathways for discarding excessive nitrogen are extensively employed, the prognosis of UCD remains unsatisfactory. Prospective clinical trials are necessary to evaluate whether supplementation with BCAAs might improve growth or neurological outcomes and decrease metabolic crisis episodes in patients with UCD.

Studies on the Effects of Caponization and Various Hormone Treatment on the Meat Production and Quality in Growing Chicken (닭에 있어서 거세(去勢) 및 Hormone 처리(處理)가 산육성(産肉性) 및 육질(肉質)에 미치는 영향(影響)에 관한 연구(硏究))

  • Ra, Kwang Yon
    • Korean Journal of Agricultural Science
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    • v.2 no.1
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    • pp.9-47
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    • 1975
  • These experiments were caried out to study the effects of caponization and various hormone treatments upon meat production and improvement of meat quality of growing chicken. Sixtyseven days old 160 New Hampshire cockerels were treated and growth rate, carcass yield, change of weight of individual organs, meat composition and change of amino acid were measured and analysed. Otherwise change of testis and thyroid gland by hormone treatment were investigated histologically. The results obtained were as follows. 1. The effectst of caponization and hormone treatment upon meat production were; 1) Body weight of cockerels in D. E. S. group without caponization was increased. upon 96.86% than initial period and A. C. T. H. group was 104.22% but other groups and all carponization groups were lighter than those of control group. 2) Weekly body gain of D. E. S. group without caponization was best showing the significance (102.69 g) and the group with caponization were lower than those groups without caponization. 3) Carcass yield was best in Testo. group without caponization (831.2 g) and the group with caponization were lower than the group without caponization. 4) Carcass rate was highest in A. C. T. H. group with caponization and (67.22%) lowest in Testo. group without caponization (63.37%), but any significance was not recognized. 2. The effects of caponizatitn and hormone treatments upon the coposition of meat and amino acids were; 1) Any significance was not recognized between treated and untreated group about change of moisture, crude protein, crude ash and glycogen contents in meat. 2) Fat co tent in muscle in the all treated groups were higher than that of control group. 3) Extracts of group without caponization were higher than those of groups with caponization. 4) Lysin contents were highest in D. E. S. group with caponization (11. 12/ 16.0 g N) and generelly Testo. group was lower compared with D. E. S. group. 5) Histidine and Arginine contents were higher in the groups with caponization than without caponization. 6) Aspartic acid content were higher in D. E. S. group and A. C. T. H. group without depend on caponization. 7) Treonine content was higher in Testo. group without caponization and in the group with caponization and without hormone treatment compared with those of control group without caponization. 8) Serine content was decreased in the group with caponization and increased by D. E. S. and A. C. T. H treatment groups and glutamic acid was also decreased in Testo. group with out caponization. 9) Cystine content was decreased by Testo. treatment and was not appeared in Testo. group without caponization. 10) Valine content was lower in control group with caponization but significance was not recognized between other groups and control group without caponization. 11) Glycine, Alanine, Methionine. Isoleucine, Leucine, Thyrosine and Phenylalanine contents were not so difference between hormone treated groups and control group without caponization. 3. The effects of caponization and hormone treatment upon the change of organs were: 1) The weight of all organs were heaviest in D. E. S. group without caponization (18.5g) and lightest in A. C. T. H. group without caponization (155. 3g) but no significance was recognized between hormone treatment groups. 2) Heart weight was heaviest in D. E. S. group without caponization (7.46 g) and lightest in Testo. group without caponization (5.95 g). 3) Liver weight was heaviest in D. E. S. group without caponization(32.89g) and lightest in hormone untreated group with caponization(29.66g). Significance was not recognized. 4) Spleen weight was heaivest in Testo. group with caponization (3.22 g) and lightest in D. E. S. group without caponization(2.00g) in contrast with the other groups. High significance was recognized among the groups (P<0.01). 5) Cloacal thymus weight was lightest in D. E. S. group with or without caponization compared with control group without caponization. High significance was recognized among the groups. 6) Muscle fat content was not appeared in A. C. T. H. group with caponization, but it was highly increased in D. E. S. group with or without caponization. 7) Testis weight was lightest in D. E. S. group (0.38g) compared with control group (2.66g). Significance was recognized among the groups. 8) Large intestine, small intestine and cecum weight and length were heavier and longer in D. E. S. group without caponization and control group without caponization was lighter than those of hormone treated groups. 4. The effects of caponization and hormone treatment upon histological change of testis and thyroid gland: 1) The histological change of testis was significantly appeared in D. E. S. group that seminifirous tubles was slowly atrophied, the funtion of spernatogenesis was ceased, spermatocyte was changed as degeneration by pyknosis and karyorrhexis and interstitial cell was also atrophied, but in Testo. and A. C. T. H. group were similar as control group. 2) The histological change of thyroid gland in Testo. and A. C. T. H. groups without caponization were similar to that of control group without caponization, but in D. E. S. group without caponization, was changed squamously. Thyroid gland of the groups with caponization, epithelium of was atrophied and changed squamously as degeneration by pyknosis and karyorrhexis and the function of thyroid gland was slowly ceased in colloid and in hormone treated group with caponization.

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Larvae Growth and Biochemical Composition Change of the Pacific Oyster Crassostra gigas, Larvae during Artificial Seed Production (참굴 Crassostrea gigas 인공종묘생산 시 유생의 성장과 체성분 변화)

  • Hur, Young-Baek;Min, Kwang-Sik;Kim, Tae-Eic;Lee, Seung-Ju;Hur, Sung-Bum
    • Journal of Aquaculture
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    • v.21 no.4
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    • pp.203-212
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    • 2008
  • A nutritional demand of oyster, Crassostrea gigas larva as part of research for improving of utilization of microalgae being used for the artificial oyster seed production. The change of body growth and biochemical compositions of larvae were investigated during larvae rearing in hatchery. The larvae were cultured in 60 M/T tank and fed mixture 6 different phytoplankton species, Isochrysis galbana (30%), Cheatoceros gracilis (20%), Pavlova lutheri (20%), Phaeodactylum triconutum (10%), Nannochryis oculata (10%) and Tetraselmis tetrathele (10%). The initial feeding amount was $0.3{\times}10^4cells/mL$ at three times a day to D-shaped larva and the feeding amount had been increased 30% gradually every two day since the larvae were raising. The larvae were developed from D shape to pediveliger stage for 12 days. The daily growth of shell length and hight were $5.8{\sim}30.8\;{\mu}m$ and $8.7{\sim}31.4\;{\mu}m$, respectively and weight gains were changed from D shape to pediveliger as follow: wet weight was $0.52{\sim}15.0\;{\mu}g/larva$, dry weight was $0.2{\sim}6.5\;{\mu}g/larva$, and ash free dry weight was $0.1{\sim}8.5\;{\mu}g/larva$. The larvae growth pattern shown a logarithmic phase from D shape to umbone stage but after that stage shown a exponential growth aspect. The crude protein, crude lipid and nitrogen free extract (NFE) of larvae during rearing periods were analyzed as $6.1{\sim}10.6%$, $0.6{\sim}1.1%$ and 1.0-2.7%, respectively. And the total amino acid contents of the larvae during rearing periods were in order as glutamic acid $1.26{\sim}2.24%$, aspartic acid $0.97{\sim}1.70%$, and methionine $0.12{\sim}0.33%$. Of the total fatty acid in the analyzed larvae, the saturated fatty acid (SSAFA) was decreased from 54.3% (D shaped larvae) to 17.1 % (pediveliger) as larvae development but the total mono-unsaturated fatty acid (${\Sigma}MOFA$) and Poly-unsaturated fatty acid (${\Sigma}PUFA$) were increased from 29.9% and 7.8% to 40.6% and 45.6%, respectively. By the way the each fatty acid of the larvae were composed of palmitic acid $9.89{\sim}36.95%$, oleic acid $12.17{\sim}32.29%$, linoleic acid $1.96{\sim}33.55%$, EPA $2.17{\sim}11.58%$ and DHA $1.95{\sim}4.51%$. As a result of this study, the larvae of oyster were demanded a various nutrients for healthy growth and the feeding control, expecially after umbone stage larvae are a rapidly growing time, is very important for success of artificial seed production.

Meat Quality and Nutritional Properties of Hanwoo and Imported New Zealand Beef (한우고기와 뉴질랜드산 냉장수입육의 육질 및 영양성분 비교)

  • Cho, Soo-Hyun;Kang, Geun-Ho;Seong, Pil-Nam;Park, Beom-Young;Jung, Seok-Geun;Kang, Sun-Moon;Kim, Young-Chun;Kim, Ji-Hee;Kim, Dong-Hun
    • Food Science of Animal Resources
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    • v.31 no.6
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    • pp.935-943
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    • 2011
  • This study was conducted to investigate the proximate composition, meat color, Warner-Bratzler shear force (WBS), cooking loss (CL), fatty acids composition, amino acid composition and mineral contents of Hanwoo beef (QG $1^+$, 1) and imported New Zealand black Angus beef with loin, strip loin, eye of round and chuck tender. The intramuscular fat contents were higher in the strip loin, loin and chuck tender of Hanwoo beef than New Zealand beef (p<0.05). Hanwoo QG 1 beef had higher Fe contents in the strip loin (30.52 mg/100g) and chuck tender (40.70 mg/100g) (p<0.05). Hanwoo beef had lower cooking loss and than those of New Zealand beef, whereas New Zealand beef had higher protein and amino acids contents (%) than their counterpart. There was no significant difference in the WBS between two origin samples except the chuck Hanwoo beef had significantly lower saturated fatty acids (SFA) and higher monounsaturated fatty acids contents than New Zealand beef (p<0.05). WBS values indicated that Hanwoo and New Zealand beef had similar tenderness in the loin, striploin and eye of round due to the longer aging periods of the New Zealand beef than Hanwoo beef during the distribution.

Quality characteristics of rice and rice starch-based Yakju (쌀 및 쌀 전분을 활용한 약주의 품질특성)

  • Kang, Ji-Eun;Kim, Jae-Woon;Choi, Han-Seok;Kim, Chan-Woo;Yeo, Soo-Hwan;Jeong, Seok-Tae
    • Food Science and Preservation
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    • v.22 no.3
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    • pp.353-360
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    • 2015
  • Yakju, a Korean traditional alcoholic beverage, is made from rice and Nuruk. In this study, the fermentation characteristics of Yakju was investigated using rice and rice starch. Ingredients was classified into raw material (rice, rice starch) and starter (enzyme supplements, modified Nuruk, traditional Nuruk, and yellow rice koji) for fermentation. The crude protein content of rice, rice starch, and starter were determined as follows (%): rice 6.69, rice starch 0.44, enzyme supplements 7.84, modified Nuruk 15.29, traditional Nuruk 14.28, and yellow rice koji 7.28. The alcohol content of rice with traditional Nuruk ($20.13{\pm}0.12%$) was higher than other Yakju. The concentration of organic acids of rice starch-based Yakju (389.83~538.34 mg%) was higher than that of rice Yakju (259.27~357.70 mg%). The concentration of nitrogen compound of rice Yakju (498.38~5976.93 ppm) was higher than that of rice starch-based Yakju (600.43~4463.79 ppm). In line with these findings, further studies will be necessary for the quality analysis of the rice, rice starch and fermented starter (enzyme supplements, modified Nuruk, traditional Nuruk and yellow rice koji).

Comparison of the Physicochemical Properties of Meat and Viscera of Dried Abalone (Haliotis discus hannai) Prepared using Different Drying Methods (건조방법에 따른 건조 전복 (Haliotis discus hannai)의 이화학적 특성 비교)

  • Park, Jeong-Wook;Lee, Young-Jae;Park, In-Bae;Shin, Gung-Won;Jo, Yeong-Cheol;Koh, So-Mi;Kang, Seong-Gook;Kim, Jeong-Mok;Kim, Hae-Seop
    • Food Science and Preservation
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    • v.16 no.5
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    • pp.686-698
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    • 2009
  • We sought basic data for product development and storage improvement of abalone. We explored drying methodologies, such as shade drying, cold air drying, and vacuum freeze drying. We also examined various physicochemical features of both meat and viscera. Raw abalone meat had $78.88{\pm}1.01%$ moisture, $9.24{\pm}0.27%$ crude protein, and $10.05{\pm}0.81%$ carbohydrate (all w/w). The moisture level of dried abalone meat was highest after cold air drying, at $18.38{\pm}0.91%$, and lowest after vacuum freeze drying, at $1.05{\pm}0.05%$. The total amino acid content of raw abalone meat was $17,124.05{\pm}493.18\;mg%$, and fell after shade-drying to $12,969.92{\pm}583.65\;mg%$, and to $13,328.78{\pm}653.11\;mg%$ after cold air drying. The total free amino acid content of raw abalone meat was $4,261.99{\pm}106.55\;mg%$, and rose after shade-drying to $6,336.50{\pm}285.15\;mg%$, to $5,072.04{\pm}248.53\;mg%$ after cold air drying, and to $4,638.85{\pm}218.03\;mg%$ after vacuum freeze drying. The fatty acid proportions in raw abalone meat were $47.00{\pm}0.99%$ saturated, $22.18{\pm}1.05%$ monounsaturated, and $30.82{\pm}1.45%$ polyunsaturated. In the viscera, however, the proportions were $36.72{\pm}0.74%$ saturated, $25.44{\pm}1.12%$ monounsaturated, and $37.84{\pm}1.67%$ polyunsaturated. The contents of chondroitin sulfate in raw abalone were $11.95{\pm}0.35%$ in meat and $7.71{\pm}0.19%$ in viscera (both w/w). After shade-drying, the chondroitin sulfate content was $16.57{\pm}0.90%$ in meat and $9.24{\pm}0.50%$ in viscera. The figures after cold air drying were $16.17{\pm}0.79%$ and $12.44{\pm}0.61%$, and those after vacuum freeze drying $25.17{\pm}1.16%$ and $15.22{\pm}0.70%$ (thus including the highest meat content). The level of collagen in raw abalone was $69.80{\pm}3.07\;mg/g$ in meat and $40.62{\pm}1.79\;mg/g$ in viscera. Meat and viscera dried in the shade had $144.05{\pm}7.78\;mg/g$ and $44.16{\pm}2.39\;mg/g$ collagen, respectively, whereas the figures after cold air drying were $133.29{\pm}6.53\;mg/g$ and $69.20{\pm}3.39\;mg/g$, and after vacuum freeze drying $137.51{\pm}6.33\;mg/g$ and $60.61{\pm}2.79\;mg/g$. Volatile basic nitrogen values of raw abalone showed a higher content in viscera, at $19.01{\pm}0.84\;mg%$, compared to meat ($10.10{\pm}0.44\;mg%$). The value for shade-dried abalone meat was $136.77{\pm}7.37\;mg%$ and that of viscera $197.97{\pm}10.69\;mg%$. After cold air drying the meat and visceral values were $27.32{\pm}1.34\;mg%$ and $71.37{\pm}3.50\;mg%$, respectively.

Studies on the physio-chemical properties and the cultivation of oyster mushroom(Pleurotus ostreatus) (느타리버섯의 생리화학적성질(生理化學的性質) 및 재배(栽培)에 관(關)한 연구(硏究))

  • Hong, Jai-Sik
    • Applied Biological Chemistry
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    • v.21 no.3
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    • pp.150-184
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    • 1978
  • Nutritional characteristics and physio-chemical properties of mycelial growth and fruitbody formation of oyster mushroom(Pleurotus ostreatus)in synthetic media, the curtural condition for the commerical production in the rice straw and poplar sawdust media, and the changes of the chemical components of the media and mushroom during the cultivation were investigated. The results can be summarized as follows: 1. Among the carbon sources mannitol and sucrose gave rapid mycelial growth and rapid formation of fruit-body with higher yield, while lactose and rhamnose gave no mycelial growth. Also, citric acid, succinic acid, ethyl alcohol and glycerol gave poor fruit-body formation, and acetic acid, formic acid, fumaric acid, n-butyl alcohol, n-propyl alcohol and iso-butyl alcohol inhibited mycelial growth. 2. Among the nitrogen sources peptone gave rapid mycelial growth and rapid formation of fruit-body with higher yield, while D,L-alanine, asparatic acid, glycine and serine gave very poor fruit-body formation, and nitrite nitrogens, L-tryptophan and L-tyrosine inhibited mycelial growth. Inorganic nitrogens and amino acids added to peptone were effective for fruit-body growth, and thus addition of ammonium sulfate, ammonium tartarate, D,L-alanine and L-leucine resulted in about 10% increase fruit-body yield. L-asparic acid about 15%, L-arginine about 20%, L-glutamic acid, and L-lysine about 25%. 3. At C/N ratio of 15.23 fruit-body formation was fast, but the yield decreased, and at C/N ratio of 11.42 fruit-body formation was slow, but the yield increased. Also, at the same C/N ratio the higher the concentration of mannitol and petone, the higher yield was produced. Thus, from the view point of both yield of fruit-body and time required for fruiting the optimum C/N ratio would be 30. 46. 4. Thiamine, potassium dihydrogen phosphate and magnecium sulfate at the concentration of $50{\mu}g%$. 0.2% and 0.02-0.03%, respectively, gave excellent mycelial and fruit-body growth. Among the micronutrients ferrous sulfate, zinc sulfate and manganese sulfate showed synergetic growth promoting effect but lack of manganese resulted in a little reduction in mycelial and fruit-body growth. The optimum concentrati on of each these nutrients was 0.02mg%. 5. Cytosine and indole acetic acid at 0.2-1mg% and 0.01mg%, respectively, increased amount of mycelia, but had no effect on yield of fruit-body. The other purine and pyrimidine bases and plant hormones also had no effect on mycelial and fruit-belly yield. 6. Illumination inhibited mycelial growth, but illumination during the latter part of vegetative growth induced primordia formation. The optimum light intensity and exposure time was 100 to 500 lux and 6-12 hours per day, respectively. Higher intensity of light was injurous, and in darkness only vegetative growth without primordia formation was continued. 7. The optimum temperature for mycelial growth was $25^{\circ}C$ and for fruit-body formation 10 to $15^{\circi}C$. The optimum pH range was from 5.0 to 6.5. The most excellent fry it-body formation were produced from the mycelium grown for 7 to 10 days. The lesser the volume of media, the more rapid the formation of fruit-body; and the lower the yield of fruit-body; and the more the volume of media, the slower the formation of fruit-body, and the higher the yield of fruit-body. The primordia formation was inhibited by $CO_2$. 8. The optimum moisture content for mycelial growth was over 70% in the bottle media of rice straw and poplar sawdust. 10% addition of rice bran to the media exhibited excellent mycelial growth and fruit-body formation, and the addition of calciumcarbonate alone was effective, but the addition of calcium carbonate was ineffective in the presence of rice bran. 9. In the cultivation experiments the total yield of mushroom from the rice straw media was $14.99kg/m^2$, and from the sawdust media $6.52kg/m^2$, 90% of which was produced from the first and second cropping period. The total yield from the rice straw media was about 2.3 times as high as that from the sawdust media. 10. Among the chemical components of the media little change was observed in the content of ash on the dry weight basis, and organic matter content decreased as the cultivation progressed. Moisture content, which was about 79% at the time of spawning, decreased a little during the period of mycelial propagation, after which no change was observed. 11. During the period from spawning to the fourth cropping about 16.7% of the dry matter, about 19.3% of organic matter, and about 40% of nitrogen were lost from the rice straw media; about 7.5% of dry mallet, about 7.6% of organic matter, and about 20% of nitrogen were lost from the sawdust media. For the production of 1kg of mushroom about 232g of organic matter and about 7.0g of nitrogen were consumed from the rice straw media; about 235g of organic matter and about 6.8g of nitrogen were consumed from the sawdust media, 1㎏ of mushroom from either of media contains 82.4 and 82.3g of organic matter and 5.6 and 5.4g of nitrogen, respectively. 12. Total nitrogen content of the two media decreased gradually as the cultivation progressed, and total loss of insoluble nitrogen was greater than that of soluble nitrogen. Content of amino nitrogen continued to increase up to the third cropping time, after which it decreased. 13. In the rice straw media 28.0 and 13.8% of the total pentosan and ${\alpha}$-cellulose, respectively, lost during the whole cultivation period was lost during the period of mycelial growth; in the sawdust media 24.1 and 11.9% of the total pentosan and ${\alpha}$-cellulose, respectively, was lost during the period of mycelial growth. Lignin content in the media began to decrease slightly from the second cropping time, while the content of reduced sugar, trehalose and mannitol continued to increase. C/N ratio of the rice straw media decreased from 33.2 at spawining to 30.0 at ending; that of the sawdust media decreased from 61.3 to 60.0. 14. In both media phosphorus, potassium, manganese and zinc decreased, at magnesium, calcium and copper showed irregular changes, and iron had a tendency to be increased. 15. Enzyme activities are much higher in the rice straw media than in the sawdust media. CMC saccharifying and liquefying activity gradually increased from after mycelial propagation to the second cropping, after which it decreased in both media. Xylanase activity rapidly and greatly increased during the second cropping period rather than the first period. At the start of the third cropping period the activity decreased rapidly in the rice straw media, which was not observed in the sawdust media. Protease activity was highest after mycelial propagation, after which it gradually decreased. The pH of the rice straw media decreased from 6.3 at spawning to 5.0 after fourth cropping; that of the sawdust media decreased from 5.7 to 4.9. 16. The contents of all the components except crude fibre of the mushroom from the rice straw media were higher than those from the sawdust media. Little change was observed in the content of the components of mushroom cropped from the first to the third period, but slight decrease was noticed at the fourth cropping.

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