Branched-chain amino acids (BCAAs) are essential amino acids that are not synthesized in our body; thus, they need to be obtained from food. They have shown to provide many physiological and metabolic benefits such as stimulation of pancreatic insulin secretion, milk production, adipogenesis, and enhanced immune function, among others, mainly mediated by mammalian target of rapamycin (mTOR) signaling pathway. After identified as a reliable marker of obesity and type 2 diabetes in recent years, an increasing number of studies have surfaced implicating BCAAs in the pathophysiology of other diseases such as cancers, cardiovascular diseases, and even neurodegenerative disorders like Alzheimer's disease. Here we discuss the most recent progress and review studies highlighting both correlational and potentially causative role of BCAAs in the development of these disorders. Although we are just beginning to understand the intricate relationships between BCAAs and some of the most prevalent chronic diseases, current findings raise a possibility that they are linked by a similar putative mechanism.
Adipogenesis ; Alzheimer Disease ; Amino Acids, Branched-Chain ; Amino Acids, Essential ; Cardiovascular Diseases ; Chronic Disease ; Heart Failure ; Insulin ; Metabolism ; Milk ; Neurodegenerative Diseases ; Obesity ; Sirolimus

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

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

There have been reports that hyperammonemia and amino acid disturbance can cause loss of consciousness in patients with multiple myelomas and normal liver function. We experienced a case of a 71-years-old female patient with amyloidosis, who had shown disturbance of consciousness. At that time, the serum ammonia level was elevated; serum amino acids disturbance was also noted. In particular, the decrease in branched-chain amino acids and increase in aromatic amino acids results in a low Fisher ratio. The Fisher ratio, the ratio of branched-chain to aromatic amino acids, has been suggested as an important indicator of consciousness disturbance. We report, for the first time in Korea, a case of amyloidosis, with mental disturbance due to serum amino acid disturbance.
Amino Acids ; Amino Acids, Aromatic ; Amino Acids, Branched-Chain ; Ammonia ; Amyloidosis* ; Consciousness ; Female ; Humans ; Hyperammonemia* ; Korea ; Liver ; Multiple Myeloma ; Unconsciousness

Vilacid has 21 amino acids, in which has essential amino acids that help treatment for malnutrition in children, especially treat for children with dyspepsia. Clinical trial treated children of malnutrition suggest that its effect is to increase weight, subcutaneous fat, albumin and serum protein
Malnutrition ; Amino Acids, Essential ; therapeutics

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

inhibitor, 2-aminobicyclo-(2,2,1)-heptane-2-carboxylic acid (BCH). The affinity of [14C]L-leucine uptake and the inhibition profiles of [14C]L-leucine uptake by various amino acids in the Saos2 cells were comparable with those for the LAT1 expressed in Xenopus oocytes. The majority of [14C]Lleucine uptake is, therefore, mediated by LAT1 in the Saos2 cells. These results suggest that the transports of neutral amino acids including several essential amino acids into Saos2 human osteogenic sarcoma cells are for the most part mediated by LAT1. Therefore, the Saos2 human osteogenic sarcoma cells are excellent tools for examine the properties of LAT1. Moreover, the specific inhibition of LAT1 in tumor cells might be a new rationale for anti-tumor therapy.
Amino Acid Transport System L* ; Amino Acids ; Amino Acids, Essential ; Amino Acids, Neutral ; Humans* ; Oocytes ; Osteosarcoma* ; Xenopus

OBJECTIVETo analyze and summarize clinical manifestation of maple syrup urine disease (MSUD) of neonates.

METHODSData of two cases with neonatal MSUD and the reports of 15 cases seen in the past 15 years in China were reviewed and analyzed.

RESULTSThere was an increasing number of reports of cases with neonatal MSUD. All the 17 cases had the symptom of poor feeding between 3 h and 8 d after birth; 7 cases had family history; 14 cases showed progressive neurologic signs. Odor of maple syrup occurred in 8 cases. Blood levels of branched-chain amino acids (BCAA) significantly increased in 13 cases and 6 neonates were diagnosed using tandem mass spectrometry. Urinary levels of BCAA and metabolite elevated in 12 cases and 5 neonates were diagnosed using gas chromatography-mass spectrometry. MRI/CT demonstrated abnormal signal in 10 cases. Twelve cases died or their parents gave up treatment and one case had cerebral palsy; 4 cases were treated with BCAA-free formula milk and showed improved outcome.

CONCLUSIONNewborns with MSUD often had early appeared non-specific symptoms with poor feeding and lethargy, most cases later showed an odor resembling maple syrup and neurologic signs. For patients who were suspected of having MSUD, blood and urine concentrations of BCAA should be tested for early diagnosis. Specific MRI edema signal from brain suggests the possibility of MSUD. Early intervention and treatment after diagnosis, with compliance of parents, would improve the patient's outcome.

As a branched chain amino acid, L-valine is widely used in the medicine and feed sectors. In this study, a microbial cell factory for efficient production of L-valine was constructed by combining various metabolic engineering strategies. First, precursor supply for L-valine biosynthesis was enhanced by strengthening the glycolysis pathway and weakening the metabolic pathway of by-products. Subsequently, the key enzyme in the L-valine synthesis pathway, acetylhydroxylate synthase, was engineered by site-directed mutation to relieve the feedback inhibition of the engineered strain. Moreover, promoter engineering was used to optimize the gene expression level of key enzymes in L-valine biosynthetic pathway. Furthermore, cofactor engineering was adopted to change the cofactor preference of acetohydroxyacid isomeroreductase and branched-chain amino acid aminotransferase from NADPH to NADH. The engineered strain C. glutamicum K020 showed a significant increase in L-valine titer, yield and productivity in 5 L fed-batch bioreactor, up to 110 g/L, 0.51 g/g and 2.29 g/(L‧h), respectively.
Valine ; Corynebacterium glutamicum/genetics* ; Metabolic Engineering ; Amino Acids, Branched-Chain ; Bioreactors

Amino-acid neurotransmitter system dysfunction plays a major role in the pathophysiology of depression. Several studies have demonstrated the potential of amino acids as a source of neuro-specific biomarkers could be used in future diagnosis of depression. Only partial amino acids such as glycine and asparagine were determined from certain parts of rats' brain included hippocampi and cerebral cortex in previous studies. However, according to systematic biology, amino acids in different area of brain are interacted and interrelated. Hence, the determination of 34 amino acids through entire rats' brain was conducted in this study in order to demonstrate more possibilities for biomarkers of depression by discovering other potential amino acids in more areas of rats' brain. As a result, 4 amino acids (L-aspartic acid, L-glutamine, taurine and gamma-amino-n-butyric acid) among 34 were typically identified as potentially primary biomarkers of depression by data statistics. Meanwhile, an antidepressant called Fluoxetine was employed to verify other potential amino acids which were not identified by data statistics. Eventually, we found L-alpha-amino-adipic acid could also become a new potentially secondary biomarker of depression after drug validation. In conclusion, we suggested that L-aspartic acid, L-glutamine, taurine, gamma-amino-n-butyric acid and L-alpha-amino-adipic acid might become potential biomarkers for future diagnosis of depression and development of antidepressant.
Amino Acids ; Animals ; Asparagine ; Aspartic Acid ; Biomarkers ; Biology ; Brain* ; Cerebral Cortex ; Depression ; Diagnosis ; Fluoxetine ; Glutamine ; Glycine ; Neurotransmitter Agents ; Rats* ; Taurine