scholarly journals Large Neutral Amino Acids (LNAAs) Supplementation Improves Neuropsychological Performances in Adult Patients with Phenylketonuria

Nutrients ◽  
2020 ◽  
Vol 12 (4) ◽  
pp. 1092 ◽  
Author(s):  
Iris Scala ◽  
Maria Pia Riccio ◽  
Maria Marino ◽  
Carmela Bravaccio ◽  
Giancarlo Parenti ◽  
...  
Keyword(s):  
Amino Acids ◽  
Executive Functions ◽  
Metabolic Control ◽  
Cognitive Functions ◽  
Well Being ◽  
Wisconsin Card Sorting Test ◽  
Card Sorting ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids ◽  
Poor Metabolic Control

Phenylketonuria is an inborn error of phenylalanine (Phe) metabolism diagnosed by newborn screening and treated early with diet. Although diet prevents intellectual disability, patients often show impairment of executive functions, working memory, sustained attention, and cognitive flexibility. Large neutral amino acids (LNAAs) have been proposed as a dietary supplement for PKU adults. Few studies show that LNAAs may help in improving metabolic control as well as cognitive functions. In this study, 10 adult PKU patients with poor metabolic control were treated for 12 months with LNAAs (MovisCom, 0.8–1 g/kg/day) and underwent Phe and Tyrosine (Tyr) monitoring monthly. Neuropsychological assessment was performed at T0, T+3, and T+12 months by using the American Psychological General Well-Being Index, the Wisconsin Card Sorting Test, the Test of Attentional Performance, and the 9-Hole Peg Test. No change in plasma Phe levels was observed during LNAAs supplementation, while Tyr levels significantly improved during LNAAs supplementation (p = 0.03). Psychometric tests showed an improvement of distress and well-being rates, of executive functions, attention, and vigilance, whereas no difference was noted regarding hand dexterity. This study adds evidence of the advantage of LNAAs supplementation in improving cognitive functions and well-being in patients with PKU with poor metabolic control.

Dopaminergic Genes Polymorphisms and Prefrontal Cortex Efficiency Among Obese People - Whether Gender is a Differentiating Factor?

2019 ◽  
Vol 19 (6) ◽  
pp. 405-418
Author(s):  
Maciej Bieliński ◽  
Natalia Lesiewska ◽  
Roman Junik ◽  
Anna Kamińska ◽  
Andrzej Tretyn ◽  
...  
Keyword(s):  
Prefrontal Cortex ◽  
Executive Functions ◽  
Tandem Repeats ◽  
Wisconsin Card Sorting Test ◽  
Variable Number ◽  
Card Sorting ◽  
Obese People ◽  
Perseverative Errors ◽  
Wisconsin Card Sorting ◽  
Sorting Test

Background:Obesity is a chronic condition associated with poorer cognitive functioning. Wisconsin Card Sorting Test (WCST) is a useful tool for evaluating executive functions. In this study, we assessed the association between dopaminergic gene polymorphisms: DAT1 (SLC6A3), COMTVal158Met, DRD4 (48-bp variable number of tandem repeats - VNTR) and WCST parameters to investigate the functions of the frontal lobes in obese individuals.Objective:To find the significant correlations between polymorphisms of DAT1, COMTVal158Met, DRD4 and executive functions in obese subjects.Methods:The analysis of the frequency of individual alleles was performed in 248 obese patients (179 women, 69 men). Evaluation of the prefrontal cortex function (operating memory and executive functions) was measured with the Wisconsin Card Sorting Test (WCST). Separate analyzes were performed in age subgroups to determine different activities and regulation of genes in younger and older participants.Results:Scores of WCST parameters were different in the subgroups of women and men and in the age subgroups. Regarding the COMT gene, patients with A/A and G/A polymorphisms showed significantly better WCST results in WCST_P, WCST_CC and WCST_1st. Regarding DAT1 men with L/L and L/S made less non-perseverative errors, which was statistically significant. In DRD4, significantly better WCST_1st results were found only in older women with S allele.Conclusion:Obtained results indicate the involvement of dopaminergic transmission in the regulation of prefrontal cortex function. Data analysis indicates that prefrontal cortex function may ensue, from different elements such as genetic factors, metabolic aspects of obesity, and hormonal activity (estrogen).

Atypical amino acids inhibit histidine, valine, or lysine transport into rat brain

1983 ◽  
Vol 245 (4) ◽  
pp. R556-R563 ◽  
Author(s):  
J. K. Tews ◽  
A. E. Harper
Keyword(s):  
Amino Acids ◽  
Rat Brain ◽  
Blood Brain Barrier ◽  
Brain Slices ◽  
Aromatic Amino Acids ◽  
Brain Barrier ◽  
Basic Amino Acids ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids ◽  
Single Meal

Transport of histidine, valine, or lysine into rat brain slices and across the blood-brain barrier (BBB) was determined in the presence of atypical nonprotein amino acids. Competitors of histidine and valine transport in slices were large neutral amino acids including norleucine, norvaline, alpha-aminooctanoate, beta-methylphenylalanine, and alpha-aminophenylacetate. Less effective were aromatic amino acids with ring substituents; ineffective were basic amino acids and omega-amino isomers of norleucine and aminooctanoate. Lysine transport was moderately depressed by homoarginine or ornithine plus arginine; large neutral amino acids were also similarly inhibitory. Histidine or valine transport across the BBB was also strongly inhibited by large neutral amino acids that were the most effective competitors in the slices (norvaline, norleucine, alpha-aminooctanoate, and alpha-aminophenylacetate); homoarginine and 8-aminooctanoate were ineffective. Homoarginine, ornithine, and arginine almost completely blocked lysine transport, but the large neutral amino acids were barely inhibitory. When rats were fed a single meal containing individual atypical large neutral amino acids or homoarginine, brain pools of certain large neutral amino acids or of arginine and lysine, respectively, were depleted.

scholarly journals Contribution to the evaluation of language disturbances in subcortical lesions: a piloty study

2004 ◽  
Vol 62 (1) ◽  
pp. 51-57 ◽  
Author(s):  
Márcia Radanovic ◽  
Letícia Lessa Mansur ◽  
Mariana Jardim Azambuja ◽  
Cláudia Sellitto Porto ◽  
Milberto Scaff
Keyword(s):  
Executive Functions ◽  
Cognitive Abilities ◽  
Wisconsin Card Sorting Test ◽  
Cognitive Networks ◽  
Vascular Lesions ◽  
Card Sorting ◽  
Subcortical Structures ◽  
Boston Naming Test ◽  
Language Abilities ◽  
Trail Making

Subcortical structures are in a strategic functional position within the cognitive networks and their lesion can interfere with a great number of functions. In this study, we describe fourteen subjects with exclusively subcortical vascular lesions (eight in the basal ganglia and six in the thalamus) and the interrelation between their language alterations and other cognitive abilities, as attention, memory and frontal executive functions. All patients were evaluated through the following batteries: Boston Diagnostic Aphasia Examination, Boston Naming Test, Token Test, Benton Visual Retention Test, Trail Making, Wisconsin Card Sorting Test and a frontal scripts task. All patients underwent MRI and twelve underwent SPECT. Results show that these patients present impairment in several cognitive domains, especially attention and executive functions. These alterations affect language abilities, and this fact must be considered in the rehabilitation efforts.

scholarly journals Trans-placental Gradients of Large Neutral Amino Acids (LNAA) in Baboon Pregnancy With Fasting,Feeding and Tryptophan (TRP) Infusion † 298

1998 ◽  
Vol 43 ◽  
pp. 53-53
Author(s):  
S Rastogi ◽  
K Abildskov ◽  
S S Daniel ◽  
M Garland ◽  
K Schulze ◽  
...  
Keyword(s):  
Amino Acids ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids

Acute effects of aspartame on large neutral amino acids and monoamines in rat brain

Life Sciences ◽  
1983 ◽  
Vol 32 (14) ◽  
pp. 1651-1658 ◽  
Author(s):  
John D. Fernstrom ◽  
Madelyn H. Fernstrom ◽  
Marcia A. Gillis
Keyword(s):  
Amino Acids ◽  
Rat Brain ◽  
Acute Effects ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids

Large neutral amino acids in the treatment of phenylketonuria (PKU)

2006 ◽  
Vol 29 (6) ◽  
pp. 732-738 ◽  
Author(s):  
R. Matalon ◽  
K. Michals-Matalon ◽  
G. Bhatia ◽  
E. Grechanina ◽  
P. Novikov ◽  
...  
Keyword(s):  
Amino Acids ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids

scholarly journals Clinical and neuropsychological assessment of executive function in a sample of children and adolescents with idiopathic epilepsy

2014 ◽  
Vol 72 (12) ◽  
pp. 954-959 ◽  
Author(s):  
Andrea Bandeira de Lima ◽  
Fernanda Moreira ◽  
Marleide da Mota Gomes ◽  
Heber Maia-Filho
Keyword(s):  
Academic Performance ◽  
Executive Function ◽  
Executive Functions ◽  
Children And Adolescents ◽  
Clinical Data ◽  
Wisconsin Card Sorting Test ◽  
Idiopathic Epilepsy ◽  
Control Group ◽  
Seizure Type ◽  
Card Sorting

Objective To compare the executive functions of children and adolescents with idiopathic epilepsy with a control group and to correlate with clinical data, intelligence and academic performance. Method Cross-sectional, descriptive and analytical study. Thirty-one cases and thirty-five controls were evaluated by the WCST (Wisconsin Card Sorting Test).The results were compared with clinical data (seizure type and frequency, disease duration and number of antiepileptic drugs used), IQ (WISC-III) and academic performance (APT). Results Patients with epilepsy had poorer executive function scores. There was no positive linear correlation between test scores and epilepsy variables. There was a positive association between academic performance and some executive function results. Conclusion Children with well controlled idiopathic epilepsy may show deficits in executive functions in spite of clinical variables. Those deficits may influence academic performance.

scholarly journals Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria

Nutrients ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 4012
Author(s):  
Iris Scala ◽  
Daniela Concolino ◽  
Anna Nastasi ◽  
Giulia Esposito ◽  
Daniela Crisci ◽  
...  
Keyword(s):  
Amino Acids ◽  
Slow Release ◽  
Good Adherence ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids ◽  
Beneficial Effects ◽  
Adherence To Diet ◽  
Low Protein ◽  
Single Oral Administration ◽  
Stable Plasma

The mainstay of phenylketonuria treatment is a low protein diet, supplemented with phenylalanine (Phe)-free protein substitutes and micronutrients. Adhering to this diet is challenging, and even patients with good metabolic control who follow the dietary prescriptions in everyday life ignore the recommendations occasionally. The present study explores the ability of slow-release large neutral amino acids (srLNAAs) to prevent Phe increase following a Phe dietary load. Fourteen phenylketonuric patients aged ≥13 years were enrolled in a 6-week protocol. Oral acute Phe loads of 250 and 500 mg were added to the evening meal together with srLNAAs (0.5 gr/kg). Phe and tyrosine were dosed before dinner, 2h-after dinner, and after the overnight fast. After oral Phe loads, mean plasma Phe remained stable and below 600 µmol/L. No Phe peaks were registered. Tyrosine levels significantly increased, and Phe/Tyrosine ratio decreased. No adverse events were registered. In conclusion, a single oral administration of srLNAAs at the dose of 0.5 gr/kg is effective in maintaining stable plasma Phe during acute oral loads with Phe-containing food and may be added to the dietetic scheme in situations in which patients with generally good adherence to diet foresee a higher than prescribed Phe intake due to their commitments.

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