Ammonium metabolism in humans

Adeva, María M.; Souto, Gema; Blánco, N.; Donapetry, Cristóbal

doi:10.1016/j.metabol.2012.07.007

Cited by 235 publications

(191 citation statements)

References 165 publications

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“…Many of these can affect brain function, causing hyperammonaemic encephalopathy and can be fatal. This is particularly critical in many disorders such as hyperammonaemia of the new-born, inborn errors of metabolism including urea cycle defects, organic acidaemias, hyperinsulinism/hyperammonaemia and liver disease [2]. There has been an increase in research efforts around electrochemical sensors for healthcare use due to increases in population and lifespan along with the need to reduce ever-increasing healthcare costs [3].…”

Section: Introductionmentioning

confidence: 99%

An electrochemical sensor device for measuring blood ammonia at the point of care

Brannelly

Killard

2017

Talanta

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Section: Introductionmentioning

confidence: 99%

An electrochemical sensor device for measuring blood ammonia at the point of care

Brannelly

Killard

2017

Talanta

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“…Ammonia is a metabolic waste product produced mainly in the gut, kidney, and skeletal muscle during exercise (1 ). It is generated from microbial metabolism, deamination of amino acids, nucleic acid degradation, and protein catabolism.…”

Section: Discussionmentioning

confidence: 99%

Critically High Plasma Ammonia in an Adolescent Girl

et al. 2016

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A 12-year-old female patient presented with nonspecific symptoms including fatigue, fever, and headache persisting for approximately 4 months. Plasma ammonia was 230 g/dL (135 mol/L) (critical value Ͼ187 g/dL or 110 mol/L). The patient was referred to a pediatric emergency center for urgent evaluation of hyperammonemia. Plasma and urine amino acid testing was performed to investigate the possibility of a urea cycle disorder; however, all results were normal. Over the following 2 weeks, the patient was treated with intravenous sodium benzoate/sodium phenylacetate and L-arginine to reduce the blood ammonia concentration. However, the patient's ammonia concentration never dropped below 94 g/dL (55 mol/L) (upper limit of reference interval, 80 g/dL or 47 mol/L), with concentrations as high as 296 g/dL (174 mol/L) with inconsistent associations between ammonia concentrations and headaches. During the course of treatment, the patient suffered substantial side effects of intravenous therapy and underwent numerous costly clinical and laboratory investigations. Amino acid investigations were repeated but results remained normal. Given the large fluctuations in blood ammonia concentrations with inconsistent response to intravenous medications, the metabolic service contacted the laboratory to inquire about a possible preanalytical or analytical cause. One week before this query, a community physician also contacted the laboratory regarding hyperammonemia in several patients. However, no obvious preanalytical or analytical errors were discovered despite numerous audits of preanalytical conditions and review of quality control data.

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“…ARG isoforms are encoded by homologous genes that are mapped in distinct chromosomes (ARG 1 in chromosome 6q23 and ARG 2 in 14q24) [27,36,[53][54][55][56]. A genetic sequencing study that was performed with human kidney tissue detected that the ARG 2 sequence was 58 % homologous to that of ARG 1 [49], whereas human and mouse ARG 1 have 87 % of the sequence in common [27].…”

Section: Methodsmentioning

confidence: 99%

“…ARG 1 is the largest fraction of the total ARG expressed in the organism [26]. It is present in the cytosol of liver cells, where it is an integrated part of the urea cycle (conversion of the L-arginine substrate to L-ornithine and urea) as well as other enzymes [Nacetylglutamate synthase (NAGS), carbamoylphosphate synthetase (CPS1), mitochondrial ornithine transporter (OTC), ornithine transcarbamylase (ASL) and argininosuccinatesynthetase-1 (ASS1)] [2,47,53]. ARG 2 is mitochondrial and can be found in several tissues, mainly in the kidney.…”

Section: Methodsmentioning

confidence: 99%

Untitled

2018

Planta Med

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IntroductionSeveral diseases have been linked to the development of ED, such as atherosclerosis, hypercholesterolaemia, coronary disease, erectile dysfunction, asthma, renal failure, rheumatoid arthritis, periodontitis, psychiatric disorders, and cancer, as well as diseases with a high prevalence, such as diabetes (types 1 and 2) and SAH [1-9].The preservation of the endothelium is fundamental in maintaining the physiology of the vascular system (in the regulation of its tonus, the development of immune, structural, and proliferative functions, and interaction with other cellular types) and also in the prevention of the development/aggravation of diseases [10][11][12].

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Ammonium metabolism in humans

Cited by 235 publications

References 165 publications

An electrochemical sensor device for measuring blood ammonia at the point of care

An electrochemical sensor device for measuring blood ammonia at the point of care

Critically High Plasma Ammonia in an Adolescent Girl

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