• Journal of The Korean Society of Inherited Metabolic disease
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• v.2 no.1
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• pp.7-11
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• 2002

Isovaleric acidemia is an inborn error in metabolism due to a defect in isovaleryl-CoA dehydrogenase. Accumulation of serum isovaleric acid causes poor feeding, vomiting, lethargy, hypothermia, convulsion, mental retardation, etc. It is inherited as an autosomal recessive trait. Since the first reports of isovaleric acidemia by Tanaka et al in 1966, more than 60 cases have been reported. There are two clinically different presentations of isovaleric acidemia, with about half the patients presenting with an acute severe neonatal form and about half with a chronic intermittent form. The difference in clinical presentation may not be a consequence of differing severities of the causative mutation, but a result of the timing of application of catabolic stress or the ability to form isovalerylglycine. We described here clinical and organic acid analytical findings of in 3 cases isovaleric acidemia.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.132-136
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• 2013

Parainfluenza virus infection is one of the causes of fatal rhabdomyolysis. Rhabdomyolysis can be aggravated by mitochondrial fatty acid ${\beta}$-oxidation disorders during prolonged periods of fasting. Moreover, in patients with late-onset isovaleric acidemia, hyperammonemia may occur following catabolic stress. In the present report, we describe a case of a 4-year-old boy with parainfluenza virus infection and late-onset isovaleric acidemia that rapidly progressed to coma, seizures, and cardiorespiratory collapse. His serum ammonia and creatinine kinase (CK) levels were $385{\mu}Mol/L$ and 23,707 IU/L, respectively. Continuous renal replacement therapy (CRRT) was initiated using continuous venovenous hemodiafiltration, after which the ammonia and CK levels returned to normal. Thus, we recommend the immediate initiation of CRRT in the management of patients with life-threatening rhabdomyolysis and hyperammonemia.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.42-46
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• 2016

Isovaleric acidemia is autosomal-recessively inherited and an inborn error of metabolism caused by abnormal leucine metabolism due to the genetic defect of IVD (Isovaleryl-CoA dehydrogenase). IVD corresponds to mitochondrial matrix enzyme that acts on converting isovaleryl-CoA into 3-methylcrotonyl-CoA in the leucine catabolism. The IVD gene is located at Chromosome 15q14-q15, particularly between base pair 40,405,485 and base pair 40,435,948. It consists of 12 exons and has been reported to cause over 50 diseases so far. We conducted IVD gene test on the patient with acute isovaleric acidemia and confirmed a new type of mutation for the first time. As a result of analyzing the IVD gene sequence, we found out that c.129T>G(p.Asn43Lys) and c.1033A>G(p.Asn345Asp) mutations exist as heterozygosity at Exon 1 and Exon 10 respectively, novel mutation.

• Microbiology and Biotechnology Letters
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• v.22 no.2
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• pp.120-125
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• 1994

By using a synthetic medium simulated on the amino acid composition of soybeam, the effect of salt concentrations on the production of volatile organic acid by the strains of Zygosaccharomyces rouxii So-3101, a soy sauce yeast, was studied at the concentrations of 12.5, 18.0, 22.0 and 28.5% NaCl. The growth, consumption of glucose, and production of alcohol, total acid and volatile organic acid, showed the highest values at a concentration of 12.5% NaCl, and those values were decreased with an increase in the salt concentration. The ratio of volatile organic acid to total organic acid was remained at approximately the same level within the range of salt concentrations between 12.5~22.0%, whereas the ratio was decreased at a salt concentration of 28.5%. After incubation for 16 days, 8 volatile organic acids, i.e. acetic, propionic, n-butyric, isobutyric, isovaleric, isocaproic, n-caproic, and heptanoic acids, were detected by gas chromatography. Among the volatile organic acids, acetic acid was produced in the appreciable amiunt and its ratio to the other volatile acids was increased with an increase in the salt concentration.A small amount of isocaproic, propionic, isobutyric and isovaleric acids were produced, and n-caproic, n-butyric and heptanoic acids were detected only at the lower salt concentration.

• Journal of Life Science
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• v.24 no.12
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• pp.1325-1329
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• 2014

Gugija (Lycii chinese Miller) is traditionally consumed as a Chinese medicinal material in food, tea, or alcoholic beverages. Gugija has beneficial healthy components, but it produces an off-flavor during storage. This study compared the flavor components of fresh-dried Gugija and stale-dried Gugija. The flavor compounds in one fresh sample (sample 1) and one stale sample (sample 2) were extracted by the simultaneous distillation and extraction method. The concentrated aroma extracts were analyzed and identified by gas chromatography-mass spectrometry. Forty-five compounds, including 17 aldehydes, 8 alcohols, 6 terpene compounds, 4 esters, 3 ketones, and 3 pyrazines, were isolated in sample 1. Thirty-four compounds, including 12 aldehydes, 3 alcohols, 5 terpene compounds, 2 esters, 3 ketones, 3 pyrazines, and 1 acid, were isolated in sample 2. The main aroma components of sample 1 were 2-methyl butanal, 2-methyl propanol having sweet odor, and hexanal, (Z)-3-hexenol having grass odor, and phenyl acetaldehyde, benzyl alcohol having floral odor, and alkyl pyrazines having nutty odor. These compounds were decreased in sample 2, and several compounds containing isovaleric acid, which has a disagreeable, rancid-cheese odor were found newley.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.57-61
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• 2016

Isovaleric acidemia (IVA) is an autosomal recessively inherited organic acid disorder due to a defect of the enzyme isovaleryl-CoA dehydrogenase in the leucine metabolic pathway. Deficiency of this enzyme results in the accumulation of derivatives of isovaleryl-CoA. In acute illness in IVA, isovaleric acid and its derivatives accumulate and profound metabolic acidosis with ketosis, characteristic pungent body odor, hypoglycemia, and hyperammonemia can be developed. Additionally, recurrent vomiting, failure to thrive, developmental delay, epilepsy and mental retardation are chronic presenting symptoms and signs for IVA. On the result of newborn screening for inherited metabolic disorders, increased levels of isovalerylcarnitine (C5) are shown. However, C5 elevation can be accompanied with short/branched-chain acyl-CoA dehydrogenase (SBCAD) and therapy with certain antibiotics containing pivalic acid. Quantitative measurement of organic acids in urine and acylcarnitine profiles in plasma are necessary to differential diagnosis. Molecular genetic analysis of the IVD gene for IVA and ACADSB is also helpful to confirm IVA and SBCAD deficiency, respectively. Considering that IVA can be associated with significant morbidity and mortality at acute presentation of metabolic crisis, early diagnosis prior to the onset of symptoms by newborn screening enable to introduction of early treatment and prevention of acute and chronic complications.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.1
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• pp.61-64
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• 2017

Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, "the odor of sweaty feet," abdominal pain, vomiting, feeding intolerance, shock and coma. Recurrent acute pancreatitis associated with IVA have been rarely reported. Herein; we report a child who admitted with recurrent acute pancreatic attacks and had the final diagnosis of IVA. Mutation analysis revealed a novel homozygous mutation of (p.E117K [c.349G>A]) in the IVA gene. Organic acidemias must kept in mind in the differential diagnosis of recurrent acute pancreatic attacks in children.

• Korean Journal Plant Pathology
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• v.2 no.2
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• pp.82-88
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• 1986

Five antifungal substances were isolated from the long-term storaged extract of common purslane, and identified as isobutyric, butyric, isovaleric, valerie and caproic acids belonging to short-chain fatty acids (C4­C6). Each of these fatty acids showed more or less antifungal potency against spore germination and mycelial growth of Alternaria alternata Japanese pear pathotype in vitro. Antifungal potency of each fatty acid against spore germination was greater than that against the mycelial growth. No one of these fatty acids completely inhibited the mycelial growth at concentration lower than 200 ppm, while 50 ppm of caproic acid and 200 ppm of valerie acid completely inhibited the spore germination. The results of bioassay also suggested that chain-length of the fatty acids might be related with the antifungal potency, since fatty acids with longer chain showed higher antifungal potency.

• Animal Bioscience
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• v.35 no.9
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• pp.1379-1389
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• 2022

Objective: This study identified the major lactic acid bacteria (LAB) strains from different fermented total mixed rations (FTMRs) via metataxonomic analysis and evaluated the ability of their standard strain as ensiling inoculants for corn stover silage. Methods: The bacterial composition of eight FTMRs were analyzed by 16S rDNA sequencing. Corn stover was ensiled without LAB inoculation (control) or with 1×10⁶ cfu/g LAB standard strain (Lactobacillus vaginalis, Lactobacillus reuteri, Lactobacillus helveticus, or Lactobacillus paralimentarius) selected from the FTMRs or 10 g/t commercial silage inoculant (CSI) around 25℃ for 56 days. For each inoculation, a portion of the silage was sampled to analyze ensiling characteristics at time intervals of 0, 1, 3, 7, 14, 28, and 56 days, gas production (GP), microbial crude protein and volatile fatty acids as the measurements of rumen fermentation characteristics were evaluated in vitro with the silages of 56 days after 72 h incubation. Results: Lactobacillus covered >85% relative abundance of all FTMRs, in which L. pontis, L. vaginalis, L. reuteri, L. helveticus, and L. paralimentarius showed >4% in specific FTMRs. CSI, L. helveticus, and L. paralimentarius accelerated the decline of silage pH. Silage inoculated with L. paralimentarius and CSI produced more lactic acid the early 14 days. Silage inoculated with L. paralimentarius produced less acetic acid and butyric acid. For the in vitro rumen fermentation, silage inoculated with CSI produced more potential GP, isobutyric acid, and isovaleric acid; silage inoculated with L. helveticus produced more potential GP and isovaleric acid, silage inoculated with L. paralimentarius or L. reuteri produced more potential GP only. Conclusion: The standard strain L. paralimentarius (DSM 13238) is a promising ensiling inoculant for corn stover silage. The findings provide clues on strategies to select LAB to improve the quality of silage.

• Korean Journal Plant Pathology
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• v.2 no.2
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• pp.89-95
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• 1986

The five short-chain fatty acids such as isobutyric(C-4), butyric(C-4), isovaleric(C-5), valeric(C-5) and caproic (C-6) acids obtained from the extract of common purslane showed wide antifungal spectra against spore germination and mycelial growth of the twenty five phytopathogenic fungi tested in vitro, although there were differences in antifungal potency among them. The antifungal potency of each fatty acid varied significantly against different fungi in spore germination and mycelial growth. The seventeen fungi used for spore germination test and the sixteen fungi used for mycelial growth test can be divided into three groups depending upon differences in minimal inhibitory concentration of each fatty acid for them, respectively. Caproic acid was significantly more toxic to germination than to mycelial growth of the test fungi, while the other four fatty acids did not show such a significant differences in toxicity with a few of exceptions as shown in valerie acid. The longer the chain-length of fatty acid was, the higher the antifungal potency was shown. The normal fatty acids such as butyric and valerie acid were more toxic than their isomers to spore germination and mycelial growth of the test fungi. Each fatty acid was more toxic to spore germination of obligate parasites and some of facultative parasites, and mycelial growth of facultative parasites than to spore germination and mycelial growth of saprophytes, respectively.