scholarly journals Amino Acids Profiling for the Diagnosis of Metabolic Disorders

2020 ◽  
Author(s):  
Yana Sandlers
Keyword(s):  
Amino Acids ◽  
Metabolic Disorders

Free amino acids in extracts of cultured skin fibroblasts from patients with various amino acid metabolic disorders

2008 ◽  
Vol 7 (5) ◽  
pp. 421-425 ◽  
Author(s):  
Vivian E. Shih ◽  
Roseann Mandell ◽  
Harvey L. Levy ◽  
John W. Littlefield
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Free Amino Acids ◽  
Free Amino ◽  
Metabolic Disorders ◽  
Skin Fibroblasts ◽  
Cultured Skin

scholarly journals Longitudinal Metabolomics Reveals Ornithine Cycle Dysregulation Correlates With Inflammation and Coagulation in COVID-19 Severe Patients

2021 ◽  
Vol 12 ◽  
Author(s):  
Tao Li ◽  
Nianzhi Ning ◽  
Bo Li ◽  
Deyan Luo ◽  
Enqiang Qin ◽  
...  
Keyword(s):  
Amino Acids ◽  
Disease Progression ◽  
Metabolic Disorders ◽  
Acid Metabolism ◽  
Severe Disease ◽  
Essential Amino Acids ◽  
Dynamic Changes ◽  
Metabolic Recovery ◽  
Global Pandemic ◽  
Highly Correlated

COVID-19 is a severe disease in humans, as highlighted by the current global pandemic. Several studies about the metabolome of COVID-19 patients have revealed metabolic disorders and some potential diagnostic markers during disease progression. However, the longitudinal changes of metabolomics in COVID-19 patients, especially their association with disease progression, are still unclear. Here, we systematically analyzed the dynamic changes of the serum metabolome of COVID-19 patients, demonstrating that most of the metabolites did not recover by 1–3 days before discharge. A prominent signature in COVID-19 patients comprised metabolites of amino acids, peptides, and analogs, involving nine essential amino acids, 10 dipeptides, and four N-acetylated amino acids. The levels of 12 metabolites in amino acid metabolism, especially three metabolites of the ornithine cycle, were significantly higher in severe patients than in mild ones, mainly on days 1–3 or 4–6 since onset. Integrating blood metabolomic, biochemical, and cytokine data, we uncovered a highly correlated network, including 6 cytokines, 13 biochemical parameters, and 49 metabolites. Significantly, five ornithine cycle-related metabolites (ornithine, N-acetylornithine, 3-amino-2-piperidone, aspartic acid, and asparagine) highly correlated with “cytokine storms” and coagulation index. We discovered that the ornithine cycle dysregulation significantly correlated with inflammation and coagulation in severe patients, which may be a potential mechanism of COVID-19 pathogenicity. Our study provided a valuable resource for detailed exploration of metabolic factors in COVID-19 patients, guiding metabolic recovery, understanding the pathogenic mechanisms, and creating drugs against SARS-CoV-2 infection.

scholarly journals Mechanistic Interventions of Selected Ocimum Species in Management of Diabetes, Obesity and Liver Disorders: Transformative Developments from Preclinical to Clinical Approaches

2021 ◽  
Vol 12 (1) ◽  
pp. 1304-1323
Keyword(s):  
Gene Expression ◽  
Amino Acids ◽  
Fatty Acids ◽  
Body Weight ◽  
Metabolic Disorders ◽  
Mechanisms Of Action ◽  
Liver Disorders ◽  
Ocimum Species ◽  
Preclinical And Clinical Studies ◽  
Diabetes And Obesity

Metabolic disorders are usually categorized as inborn metabolism defects, including carbohydrate metabolism deficits in enzymes, amino acids derived from proteins, and fatty acids released from lipids. A metabolic disorder, which arises from elevated body weight, diabetes, and obesity, has reached epidemic proportions in countries. This review discusses the metabolic disorders with respect to diabetes, obesity and liver disorders and their therapeutic management with selective Ocimum species. Ocimum genus contains more than 200 species and is one of the richest sources of diverse phytoconstituents, including fatty acids, saponins, flavonoids, terpenoids, phenols, tannins, etc. that are documented to be beneficial in the management of various metabolic disorders. The potential of selected Ocimum species in metabolic disorders is discussed by reviewing available preclinical and clinical studies and associated mechanisms of action and their effect on gene expression.

scholarly journals METABOLIC DISORDERS OF AMINO ACIDS AND RELATED COMPOUNDS IN HEPATOBILIARY PATHOLOGY

2019 ◽  
Vol 3 (2) ◽  
pp. 140-144
Author(s):  
S. A. Chernyak ◽  
◽  
V. M. Tsyrkunov ◽  
L. K. Chernyak ◽  
◽  
...  
Keyword(s):  
Amino Acids ◽  
Metabolic Disorders ◽  
Related Compounds

Hereditary Metabolic Diseases

2013 ◽  
pp. 227-256
Author(s):  
Frédéric Sedel ◽  
Hans H. Goebel ◽  
Douglas C. Anthony
Keyword(s):  
Amino Acids ◽  
Clinical Features ◽  
Metabolic Disorders ◽  
Metabolic Diseases ◽  
The Other ◽  
Structural Protein ◽  
Cellular Organelle ◽  
Biochemical Pathways ◽  
Hereditary Metabolic Diseases ◽  
Intermediate Metabolite

This chapter describes and illustrates the morphologic CNS changes in hereditary metabolic disorders. In some disorders, the metabolic derangements are most prominent in the cytosol and are linked to the dysfunction of a single cellular organelle. In these disorders there may be intracellular accumulation of an intermediate metabolite, resulting in a “storage disease” or accumulation of the abnormal substance within the cell. The organelles most commonly involved in these disorders are lysosomes, peroxisomes, mitochondria, and the cytoplasmic compartment. The other disorders are not linked to a specific cellular organelle. They are defined by an enzyme deficiency, the biochemical pathways involved (metabolic disorders of sugars, copper, amino acids, or structural protein), or only by morphologic/clinical features.

Free amino acid analysis of untimed and 24-h urine samples compared.

1980 ◽  
Vol 26 (13) ◽  
pp. 1804-1808 ◽  
Author(s):  
M Y Tsai ◽  
J G Marshall ◽  
M W Josephson
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Free Amino ◽  
Metabolic Disorders ◽  
Acid Metabolism ◽  
Reference Interval ◽  
Urine Samples ◽  
Inborn Errors ◽  
Urinary Amino ◽  
Nonketotic Hyperglycinemia

Abstract We measured 11 amino acids in untimed urine samples, to determine whether such samples are suited for use in diagnosis of aminoacidurias. Results for untimed samples varied by as much as 25% more than for 24-h collections when amino acid excretions were expressed in terms of urinary creatinine. Values decreased with increasing age for either type of specimen. Urinary amino acid excretions were also determined with untimed or 24-h samples from patients with cystinuria. Lowe’s syndrome, nonketotic hyperglycinemia, or phenylketonuria. In all cases studied, the amino acids diagnostic of the diseases significantly exceeded the reference interval obtained for 260 control subjects in six age categories. We conclude that untimed urine samples can be used for diagnosis of these inborn errors of amino acid metabolism, but further studies are needed to evaluate their usefulness for other metabolic disorders.

scholarly journals Amyloid like Aggregates Formed by the Self-Assembly of Proline and Hydroxyproline

2021 ◽  
Author(s):  
Bharti Koshti ◽  
Ramesh, Singh ◽  
Vivekshinh Kshtriya ◽  
Shanka Walia ◽  
Dhiraj Bhatia ◽  
...  
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Self Assembly ◽  
Metabolic Disorders ◽  
Research Work ◽  
Single Amino Acid ◽  
Dependent Manner ◽  
Force Microscopy ◽  
Aggregation Behaviour ◽  
Significant Research

<p>Single amino acid based self-assembled structures have gained a lot of interest recently owing to their pathological significance in metabolite disorders. There is plethora of significant research work which illustrate amyloid like characteristics of assemblies formed by aggregation of single amino acids like Phenylalanine, Tyrosine, Tryptophan, Cysteine and Methionine and its implications in pathophysiology of single amino acid metabolic disorders like phenylketonuria, tyrosinemia, hypertryptophanemia, cystinuria and hypermethioninemia respectively. Hence, studying aggregation behaviour of single amino acids is very crucial to assess the underlying molecular mechanism behind metabolic disorders. In this manuscript we report for the very first time the aggregation properties of non-aromatic single amino acids Hydroxy-proline and Proline. The morphologies of these were studied extensively by Optical microscopy (OM), ThT binding fluorescence microscopy, Scanning Electron Microscopy (SEM) and Atomic force microscopy (AFM). It can be assessed that these amino acids form globular structures at lower concentrations and gradually changes to tape like structures on increasing the concentration as assessed by AFM. ThT and CR binding assay reveal the aggregates do have amyloid like characteristics. Further MTT assays on SHSY5Y neural cell lines reveal cytotoxicity and the aggregates caused significant cell death in dose dependent manner. These results have important implications in understanding the pathophysiology of single amino acid disorders like Hyperprolinemia and Hydroxyprolinemia in association with amyloid diseases. The symptoms of these diseases are also accompanied by extensive neurological problems like intellectual disability, seizures and psychiatric problems which further evince amyloid like etiology for these rare in-born errors of metabolism.</p>

scholarly journals Branched-Chain Amino Acids Exacerbate Obesity-Related Hepatic Glucose and Lipid Metabolic Disorders via Attenuating Akt2 Signaling

2020 ◽  
Author(s):  
Ada Admin ◽  
Huishou Zhao ◽  
Fuyang Zhang ◽  
Dan Sun ◽  
Xiong Wang ◽  
...  
Keyword(s):  
Amino Acids ◽  
Lipid Accumulation ◽  
Metabolic Disorders ◽  
Plasma Lipid ◽  
Branched Chain Amino Acids ◽  
Hepatic Lipogenesis ◽  
Alcoholic Fatty Liver ◽  
Insulin Resistant ◽  
Hepatic Glucose ◽  
Branched Chain

Branched chain amino acids (BCAAs) are associated with the progression of obesity-related metabolic disorders, including T2DM and non-alcoholic fatty liver disease. However, whether BCAAs disrupt the homeostasis of hepatic glucose and lipid metabolism remains unknown. In this study, we observed that BCAAs supplementation significantly reduced high-fat (HF) diet-induced hepatic lipid accumulation while increasing the plasma lipid levels and promoting muscular and renal lipid accumulation. Further studies demonstrated that BCAAs supplementation significantly increased hepatic gluconeogenesis and suppressed hepatic lipogenesis in HF diet-induced obese (DIO) mice. These phenotypes resulted from severe attenuation of Akt2 signaling via mTORC1- and mTORC2-dependent pathways. BCAAs/branched-chain α-keto acids (BCKAs) chronically suppressed Akt2 activation through mTORC1 and mTORC2 signaling and promoted Akt2 ubiquitin-proteasome-dependent degradation through the mTORC2 pathway. Moreover, the E3 ligase Mul1 played an essential role in BCAAs/BCKAs-mTORC2-induced Akt2 ubiquitin-dependent degradation. We also demonstrated that BCAAs inhibited hepatic lipogenesis by blocking Akt2/SREBP1/INSIG2a signaling and increased hepatic glycogenesis by regulating Akt2/Foxo1 signaling. Collectively, these data demonstrate that in DIO mice, BCAAs supplementation resulted in serious hepatic metabolic disorder and severe liver insulin resistance: insulin failed to not only suppress gluconeogenesis but also activate lipogenesis. Intervening BCAA metabolism is a potential therapeutic target for severe insulin-resistant disease.<br>

scholarly journals Branched-Chain Amino Acids Exacerbate Obesity-Related Hepatic Glucose and Lipid Metabolic Disorders via Attenuating Akt2 Signaling

2020 ◽  
Author(s):  
Ada Admin ◽  
Huishou Zhao ◽  
Fuyang Zhang ◽  
Dan Sun ◽  
Xiong Wang ◽  
...  
Keyword(s):  
Amino Acids ◽  
Lipid Accumulation ◽  
Metabolic Disorders ◽  
Plasma Lipid ◽  
Branched Chain Amino Acids ◽  
Hepatic Lipogenesis ◽  
Alcoholic Fatty Liver ◽  
Insulin Resistant ◽  
Hepatic Glucose ◽  
Branched Chain

Branched chain amino acids (BCAAs) are associated with the progression of obesity-related metabolic disorders, including T2DM and non-alcoholic fatty liver disease. However, whether BCAAs disrupt the homeostasis of hepatic glucose and lipid metabolism remains unknown. In this study, we observed that BCAAs supplementation significantly reduced high-fat (HF) diet-induced hepatic lipid accumulation while increasing the plasma lipid levels and promoting muscular and renal lipid accumulation. Further studies demonstrated that BCAAs supplementation significantly increased hepatic gluconeogenesis and suppressed hepatic lipogenesis in HF diet-induced obese (DIO) mice. These phenotypes resulted from severe attenuation of Akt2 signaling via mTORC1- and mTORC2-dependent pathways. BCAAs/branched-chain α-keto acids (BCKAs) chronically suppressed Akt2 activation through mTORC1 and mTORC2 signaling and promoted Akt2 ubiquitin-proteasome-dependent degradation through the mTORC2 pathway. Moreover, the E3 ligase Mul1 played an essential role in BCAAs/BCKAs-mTORC2-induced Akt2 ubiquitin-dependent degradation. We also demonstrated that BCAAs inhibited hepatic lipogenesis by blocking Akt2/SREBP1/INSIG2a signaling and increased hepatic glycogenesis by regulating Akt2/Foxo1 signaling. Collectively, these data demonstrate that in DIO mice, BCAAs supplementation resulted in serious hepatic metabolic disorder and severe liver insulin resistance: insulin failed to not only suppress gluconeogenesis but also activate lipogenesis. Intervening BCAA metabolism is a potential therapeutic target for severe insulin-resistant disease.<br>

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