PURPOSE: This study aimed to determine the amino acids composition, safety and efficacy of formulas recently developed by Korean dairy companies for children with inherited metabolic disorder. METHODS: The determination of amino acids concentration was performed on eight Korean formula samples. The samples were hydrolyzed with 6N HCL or performic acid and analyzed by amino acid analyzer. RESULTS: No phenylalanine, methionine or leucine was observed in PKU-1 and PKU-2 Formulas, Methionine-Free and Leucine-Free Formula, respectively. BCAA-Free Formula was free from leucine, isoleucine, and valine and MPA Formula did not contain methionine and valine. Protein-Free formula did not include any amino acids. UCD Formula contained arginine but was free of alanine, aspartic acid, glutamic acid, glycine, histidine, proline and serine. Methionine-Free Formula contained higher amounts of cystine and tyrosine was higher in PKU-1 and 2 Formulas. The amounts of isoleucine and threonine were minimal in MPA Formula. CONCLUSION: This study confirmed that the eight special formulas, developed for the first time by a Korean dairy company for children with inherited metabolic disorder contain appropriate amino acids with proper contents. Both the total amino acid amounts and specific amino acid concentrations of the formulas were appropriate for related diseases, which could be used safely by the patients with inherited metabolic disorder. For UCD Formula that contains arginine, we suggest that arginine be removed from the formula in order to use for any urea cycle defect patients before the specific diagnosis is made.
Alanine ; Amino Acids ; Arginine ; Aspartic Acid ; Child* ; Cystine ; Diagnosis ; Glutamic Acid ; Glycine ; Histidine ; Humans ; Isoleucine ; Leucine ; Methionine ; Phenylalanine ; Proline ; Serine ; Threonine ; Tyrosine ; Urea ; Valine

BACKGROUND: Many studies document the presence of abnormalities in amino acids metabolism in chronic uremia. These abnormalities have been attributed to low protein intake, deficiency of excretory and metabolic functions of the diseased kidneys, toxic effects of uremia on the intermediary metabolism of amino acids and in dialysis patients, loss of protein and amino acids by the dialytic procedure. METHODS: This study was designed to compare anthropometric measurement, biochemical characteristics and plasma amino acid concentration between patients with end stage renal disease on maintenance hemodialysis (HD) and normal controls. A cross sectional study of overnight fasting plasma amino acids and plasma albumin, prealbumin, triglyceride (TG), cholesterol, transferrin concentration were performed on 20 hemodialysis patients and 20 normal controls, matched by age and sex. RESULTS: The concentrations of prealbumin (25.60+/-7.05 mg/dL vs 35.08+/-8.11 mg/dL, p<0.005), transferrin (158.30+/-39.66 mg/dL vs 275.50+/-55.46 mg/dL, p<0.001) were found to be lower in HD patients. No differences in albumin, cholesterol and TG were observed between the two groups. Several amino acids (taurine, cystine, phosphoserine) were found to be higher in the HD patients, while the concentrations of other five amino acids (serine, alanine, valine, leucine, tyrosine) were lowered in HD patients. No differences in nine amino acids (asparagine, glutamine, proline, glycine, methionine, isoleucine, lysine, histidine, arginine) were observed between the two groups. CONCLUSION: Our results suggest that chronic renal failure patients have malnutrition and amino acids abnormalities. To correct the amino acids abnormalities and improve nitrogen utilization in hemodialysis patients, correction of acidosis and supplementation of the diet with serine should be considered.
Acidosis ; Alanine ; Amino Acids ; Cholesterol ; Cystine ; Dialysis ; Diet ; Fasting ; Glutamine ; Glycine ; Histidine ; Humans ; Isoleucine ; Kidney ; Kidney Failure, Chronic ; Leucine ; Lysine ; Malnutrition ; Metabolism ; Methionine ; Nitrogen ; Nutritional Status* ; Plasma* ; Prealbumin ; Proline ; Renal Dialysis* ; Serine ; Serum Albumin ; Transferrin ; Triglycerides ; Uremia ; Valine

BACKGROUND: Genetic and environmental factors are known to affect the incidence and severity of asthma. Stimulation of beta2-Adrenergic Receptor (beta 2AR) results in smooth muscle relaxation, leading to decrease in resistance of airflow. The gene encoding the beta 2AR has recently been seguenced. The beta 2AR genotype at the polymorphic lociof codons 16, 27, 34, and 164 was known to cause changes in the amino acids. The relationships between the structure of the beta 2AR and its functions are being elucidated. PURPOSE: The gene encoding the beta 2AR was carried out to assess the frequency of polymorphisms in bronchial asthma, to determine wheather these polymorphisms have any relation to the severity, or nocturnal symptoms in bronchial asthma. METHOD: The subjects studied were 103 patients with bronchial asthma, which consisted of 30 mild episodic, 32 mild persistent, 17 moderate, and 24 severe asthma patients. The polymorphisms of the beta 2AR gene were detected by mutated allele specific amplification (MASA) method at the codons 16, 27, 34, and 164. RESULTS: The most frequent polymorphism was arginine 16 to glycine. The other two polymorphisms, valine 34 to methionine and glutamine 27 to glutamic acid occured in 11 and 6 patients respectively. The polymorphism of threonine 164 to isoleucine was not found in our enrolled patients. The homozygous polymorphism of beta 2AR gene was found in only arginine 16 to glycine (12.6%). The heterozygous polymorphisms of beta 2AR gene were in arginine 16 to glycine, valine 34 to methionine, and glutamine 27 to glutamic acid, as 65.1%, 10.7%, and 5.8% respectively in asthma patients. The presence of agrginine 16 to glycine heterozygous or/and homozygous polymorphism was associated in severe asthma (p=0.015), but there was no association between the other three polymorphisms and the severity of asthma. The frequency of the 182AR gene polymorphisms was no relation in nocturnal asthma as compared with non-nocturnal asthma. CONCLUSION: The arginine 16 to glycine polymorphism of the beta 2AR gene is the most frequently found in asthma patients and association with severe asthma. But there was no association between the polymorphism of the beta 2AR gene and nocturnal asthma.
Alleles ; Amino Acids ; Arginine ; Asthma* ; Codon ; Genotype ; Glutamic Acid ; Glutamine ; Glycine ; Humans ; Incidence ; Isoleucine ; Methionine ; Muscle, Smooth ; Polymorphism, Genetic* ; Relaxation ; Threonine ; Valine

This study was performed to determine the effect of dietary Na levels on plasma amino acid levels. Plasma amino acid levels were compared in 20 adult healthy women subjects who were given high Na diet (290.48 mEq/day : NaCl 17 g) or low Na diet (51.26 mEq/day : NaCl 3 g) for subsequent 6 days. Plasma essential amino acids levels were significantly decreased (36%) while plasma non-essential amino acids levels were significantly increased (22%) when subjects were given low Na diet (p < 0.001). Among essential amino acids, threonine was decreased (74%) significantly when subjects were given low Na diet (p < 0.01). Among nonessential amino acids, serine (49%), proline (20%) and aspartic acid (14%) were increased (p < 0.01), while arginine (48%) and glutamic acid (27%) were decreased (p < 0.001). In conclusion, dietary Na contents seemed to be an important factor to affect plasma amino acid levels. It would be appropriate to decrease the dietary Na intakes level considering the various clinical effects of dietary Na on the body fluid. For the patients who need low Na diet, it would be suggested that the level of dietary proteins should be carefully considered along with dietary Na manipulation.
Adult* ; Amino Acids ; Amino Acids, Essential ; Arginine ; Aspartic Acid ; Body Fluids ; Diet* ; Dietary Proteins ; Female ; Glutamic Acid ; Humans ; Plasma* ; Proline ; Serine ; Sodium* ; Threonine

BACKGROUND/AIMS: Amino acids have many physiological activities. We report the correlation between gastric emptying and gastric adaptive relaxation using tryptophan and amino acids with a straight alkyl chain, hydroxylated chain, and branched chain. Here we sought to further clarify the correlation between gastric emptying and gastric adaptive relaxation by using other amino acids. METHODS: In Sprague-Dawley rats, gastric emptying was evaluated by a breath test using [1-¹³C] acetic acid. The expired ¹³CO₂ pattern, T(max), C(max), and AUC(120min) values were used as evaluation items. Gastric adaptive relaxation was evaluated in a barostat experiment. Individual amino acids (1 g/kg) were administered orally 30 minutes before each breath test or barostat test. RESULTS: L-phenylalanine and L-tyrosine did not influence gastric emptying. All other amino acids, ie, L-proline, L-histidine, L-cysteine, L-methionine, L-aspartic acid, L-glutamic acid, L-asparagine, L-arginine, L-glutamine, and L-lysine significantly delayed and inhibited gastric emptying. L-Cysteine and L-aspartic acid significantly enhanced and L-methionine and L-glutamine significantly inhibited gastric adaptive relaxation. L-Phenylalanine moved the balloon toward the antrum, suggesting strong contraction of the fundus. T(max) showed a significant positive correlation (r = 0.709), and C(max) and AUC(120min) each showed negative correlations (r = 0.613 and 0.667, respectively) with gastric adaptive relaxation. CONCLUSION: From the above findings, it was found that a close correlation exists between gastric emptying and adaptive relaxation, suggesting that enhanced gastric adaptive relaxation inhibits gastric emptying.
Acetic Acid ; Amino Acids* ; Animals ; Arginine ; Asparagine ; Aspartic Acid ; Breath Tests ; Cysteine ; Gastric Emptying* ; Glutamic Acid ; Glutamine ; Histidine ; Lysine ; Methionine ; Phenylalanine ; Proline ; Rats ; Rats, Sprague-Dawley ; Relaxation* ; Tryptophan ; Tyrosine

Five representative local foods of Cheollabuk-do Province such as chuotang, ochuk, baekhapchuk, dasulgitang and minmulgokimaeuntang were subjected to recipe standardization and nutrient analysis. To derive a standard recipe, first a test recipe was prepared with the consideration of information obtained from literature survey, personal interview and survey of restaurant recipe. This test recipe was modified three times after sensory evaluation. Then the modified test recipe was accepted as the standard recipe when all characteristics of food was assessed over 5 points and the percentage of judges who gave a score over five exceeded 70% in seven-point hedonic scale. Actually, all characteristics of each food were judged as "satisfactory". Nutrient analysis was performed in food cooked according to the decided standard recipe. In general, it appeared that energy content was rather low. However protein contents in chuotang, ochuk and minmulgokimaeuntang were higher than the recommended value per meal. Vitamin A, vitamin C, vitamin B1, vitamin B, niacin, calcium, phosphorus and iron were rich in chwotang and minmulgokimaeuntag. Onhuk contains plenty of vitamin C, vitamin B1, vitamin B2 and the contents of vitamin A, vitamin B1, and niacin in baekhapapchuk were over the recommended values per meal. The foods contained large percentage of aspartic acid and glutamic acid, and major essential amino acids appeared to be leucine and lysine. On the other hand, major fatty acids were oleic acid, linoleic acid and plamitic acid. Among them the content of oleic acid was the highest in chuotang, ochuk and baekhapchuk, whereas linoleic acid and palmitic acid were the most rich fatty acids in baekhapchuk and dasulgitang respectively.
Amino Acids, Essential ; Ascorbic Acid ; Aspartic Acid ; Calcium ; Fatty Acids ; Glutamic Acid ; Hand ; Humans ; Iron ; Jeollabuk-do* ; Leucine ; Linoleic Acid ; Lysine ; Meals ; Niacin ; Oleic Acid ; Palmitic Acid ; Phosphorus ; Restaurants ; Riboflavin ; Thiamine ; Vitamin A ; Vitamins

Use of ketosteril is a solution of supplying the essential aminoacide without complement of nitrogen for patient so limits the hyperuremia during renal failure. In order to enhance the effect of ketosteril, the patient should take up the low protein dietary. This dietary will limit the hyperuremia. The combination ketosteril with the low protein dietary better limits the hyperuremia, currently without leading to lack of essential aminoacid
ketosteril ; Amino Acids, Essential

A 1-year-old female with maple syrup urine disease presenting with erythematous, partially eroded plaques on the trunk, anogenital area, and extremities experienced metabolic crisis. The skin lesions appeared at 11 months of age and was thought to result from amino acid imbalance secondary to erratic supplementation of specialized milk formula devoid of isoleucine, leucine, and valine. Serial urine monitoring showed persistent ketones and elevated serum leucine and valine. The patient was managed with emollients, intralipid 20%, and addition of isoleucine and valine supplements to counter the neurotoxic effect of leucine. After 8 days of proper feeding and continuous emollient application, the lesions improved and skin biopsy revealed superficial perivascular dermatitis. Although a decrease in erythema and desquamation was noted, the patient had persistent cerebral edema and continued to deteriorate.

Hepatocellular carcinoma (HCC) is the most common primary malignant tumor of the liver and the third most common cause of cancer-related death worldwide. HCC is caused by infection of hepatitis B/C virus and liver dysfunctions, such as alcoholic liver disease, nonalcoholic fatty liver disease, and cirrhosis. Amino acids are organic substances containing amine and carboxylic acid functional groups. There are over 700 kinds of amino acids in nature, but only about 20 of them are used to synthesize proteins in cells. Liver is an important organ for protein synthesis, degradation and detoxification as well as amino acid metabolism. In the liver, there are abundant non-essential amino acids, such as alanine, aspartate, glutamate, glycine, and serine and essential amino acids, such as histidine and threonine. These amino acids are involved in various cellular metabolisms, the synthesis of lipids and nucleotides as well as detoxification reactions. Understanding the role of amino acids in the pathogenesis of liver and the effects of amino acid intake on liver disease can be a promising strategy for the prevention and treatment of liver disease. In this review, we describe the biochemical properties and functions of amino acids and to review how they have been applied to treatment of liver diseases.
Alanine ; Amino Acids ; Amino Acids, Essential ; Aspartic Acid ; Carcinoma, Hepatocellular ; Fibrosis ; Glutamic Acid ; Glycine ; Hepatitis ; Histidine ; Liver Diseases ; Liver Diseases, Alcoholic ; Liver ; Metabolism ; Non-alcoholic Fatty Liver Disease ; Nucleotides ; Serine ; Therapeutic Uses ; Threonine

Methylmalonic acidemia is a rare congenital autosomal recessive metabolic disease. It is caused by blocking in the pathways of isoleucine, valine, threonine, methionine, cholesterol and odd-chain fatty acids to succinyl CoA, resulting in the increase of L-methylmalonyl CoA and methylmalonic acid. In most cases, there are symptoms such as recurrent vomitings, lethargy and laboratory abnormalities including metabolic acidosis and hyperammonemia from the neonatal period. We had a 6-month-old infant with methylmalonyl acidemia who presented with recurrent vomiting episodes since 3 months of age, failure to thrive and developmental delay. The laboratory findings showed hyperammoninemia and ketotic metabolic acidosis. Plasma amino acid analysis showed nonspecific finding. Urine organic acid ananysis by gas chromatography and mass spectrometry detected large amount of methylmalonic acid excreted in the urine. We restrained the supply of protein in the amount of 1~1.5 g/kg of body weight a day using leucine, isoleucine and valine-r-estrained milk and administered vitamine B12, in the amount of 1mg per day. During the follow-up in the outpatient clinic, He could control his head and showed increased muscle strength.
Acidosis ; Ambulatory Care Facilities ; Body Weight ; Cholesterol ; Chromatography, Gas ; Failure to Thrive ; Fatty Acids ; Follow-Up Studies ; Head ; Humans ; Hyperammonemia ; Infant* ; Isoleucine ; Lethargy ; Leucine ; Mass Spectrometry ; Metabolic Diseases ; Methionine ; Methylmalonic Acid ; Milk ; Muscle Strength ; Plasma ; Threonine ; Valine ; Vitamins ; Vomiting