Disorders of phenylalanine and tyrosine metabolism

Alsharhan, Hind; Fıçıcıoğlu, Can

doi:10.3233/trd-200049

Cited by 14 publications

(10 citation statements)

References 233 publications

(336 reference statements)

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“…Any kind of abnormality in the concentration levels of L-tyrosine can lead to several disorders, such as hypo- and hyper-thyroidism, in living beings [ 7 ]. In addition, dementia, depression, Parkinson’s disease, tyrosinemia and disfunction of the kidney and liver have also been reported due to unbalanced concentrations of L-tyrosine in living systems [ 8 , 9 ]. Additionally, increased concentrations of L-tyrosine in the body has caused atherosclerosis, joint pains and heartburn in some cases [ 10 ].…”

Section: Introductionmentioning

confidence: 99%

Coconut Carbon Dots: Progressive Large-Scale Synthesis, Detailed Biological Activities and Smart Sensing Aptitudes towards Tyrosine

et al. 2022

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In the recent era, carbon dots (C-dots) have been extensively considered as a potential tool in drug delivery analysis. However, there have been fewer reports in the literature on their application in the sensing of amino acids. As part of our ongoing research on coconut-husk-derived C-dots, we synthesized C-dots under different temperature conditions and utilized them in the field of amino acid sensing and found them to be highly selective and sensitive towards tyrosine. The detailed characterization of the prepared C-dots was carried out. The developed C-dots exhibit good values of quantum yield. BSA, HSA and glutamic acid were utilized to explore the binding efficiency of C-dots with biologically active components. Hemolysis, blood clotting index activity and cell viability assays using the prepared C-dots were evaluated and they were found to be biocompatible. Therefore, the C-dots described in this work have high potential to be utilized in the field of amino acid sensing, especially L-tyrosine. The limit of detection and the binding constant for the developed C-dots in the presence of tyrosine were found to be 0.96 nM and 296.38 nM−1, respectively. The efficiency of the developed C-dots was also investigated in the presence of various other amino acids and different water mediums in order to enhance the working scope of the developed sensors.

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Section: Introductionmentioning

confidence: 99%

Coconut Carbon Dots: Progressive Large-Scale Synthesis, Detailed Biological Activities and Smart Sensing Aptitudes towards Tyrosine

et al. 2022

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“…In HT-3, an inherited deficiency of HPPD, both of these factors may also play a part in mitigating increase in sTYR. In HT-2 on the other hand, the formation of HPLA, catalysed by an oxidoreductase called 4-hydroxyphenyllactate:NAD + oxidoreductase, also called HPPR 33 , 34 is lacking and leaves only increase in PHE driven by the increase in TYR to minimise tyrosinaemia, the reason why HT-2 patients develop more severe tyrosinaemia than HT-3 patients 35 .…”

Section: Discussionmentioning

confidence: 99%

Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria

Ranganath

Milan

Hughes

et al. 2022

Sci Rep

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Nitisinone (NIT) produces inevitable but varying degree of tyrosinaemia. However, the understanding of the dynamic adaptive relationships within the tyrosine catabolic pathway has not been investigated fully. The objective of the study was to assess the contribution of protein intake, serum NIT (sNIT) and tyrosine pathway metabolites to nitisinone-induced tyrosinaemia in alkaptonuria (AKU). Samples of serum and 24-h urine collected during SONIA 2 (Suitability Of Nitisinone In Alkaptonuria 2) at months 3 (V2), 12 (V3), 24 (V4), 36 (V5) and 48 (V6) were included in these analyses. Homogentisic acid (HGA), tyrosine (TYR), phenylalanine (PHE), hydroxyphenylpyruvate (HPPA), hydroxyphenyllactate (HPLA) and sNIT were analysed at all time-points in serum and urine. Total body water (TBW) metabolites were derived using 60% body weight. 24-h urine and TBW metabolites were summed to obtain combined values. All statistical analyses were post-hoc. 307 serum and 24-h urine sampling points were analysed. Serum TYR from V2 to V6, ranging from 478 to 1983 µmol/L were stratified (number of sampling points in brackets) into groups < 701 (47), 701–900 (105), 901–1100 (96) and > 1100 (59) µmol/L. The majority of sampling points had values greater than 900 µmol/L. sPHE increased with increasing sTYR (p < 0.001). Tyrosine, HPPA and HPLA in serum and TBW all increased with rising sTYR (p < 0.001), while HPLA/TYR ratio decreased (p < 0.0001). During NIT therapy, adaptive response to minimise TYR formation was demonstrated. Decreased conversion of HPPA to HPLA, relative to TYR, seems to be most influential in determining the degree of tyrosinaemia.

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“…Additionally, phenylalanine is converted to tyrosine by phenylalanine hydroxylase (PAH) in a healthy human subject. Tyrosine is further used as a precursor to neurotransmitters such as epinephrine, norepinephrine, and dopamine [ 34 , 35 ]. Thus, the phenylalanine metabolism plays an important role in mental health.…”

Section: Discussionmentioning

confidence: 99%

Distinct Changes in Microbiota-Mediated Intestinal Metabolites and Immune Responses Induced by Different Antibiotics

Yoon

Lee

et al. 2022

Antibiotics

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The cocktails of antibiotics are utilized to study the functions of microbiota. There have been studies on the alteration of not only the microbiota composition but also the host’s metabolism or immunity. However, the bacterial species associated with these altered physiologic markers are still unclear. Therefore, we supplied mice with drinking water containing ampicillin (AMP), vancomycin (VAN), neomycin (NEO), or metronidazole (MET) to observe the effect of each antibiotic on helper T cells and inflammation-related gene expression and metabolism, including amino acid metabolism and changes in gut microbiota. We observed major changes in gut microbiota in mice treated with AMP and VAN, respectively, immediately after administration. The abundance of the genera Parabacteroides and Akkermansia increased in the AMP and VAN groups, while Prevotella almost disappeared from both groups. The compositional changes in intestinal metabolites in the AMP and VAN groups were more distinct than those in the NEO and MET groups, which was similar to the microbiome results. In particular, the most distinct changes were observed in amino acid related metabolism in AMP and VAN groups; the amounts of phenylalanine and tyrosine were increased in the AMP group while those were decreased in the VAN group. The changed amounts of intestinal amino acids in each of the AMP and VAN groups were correlated with increases in the abundance of the genera Parabacteroides and Akkermansia in the AMP and VAN groups, respectively. The most distinctive changes in intestinal gene expression were observed in the ileum, especially the expression Th17-related genes such as rorgt, il17a, and il17f, which decreased dramatically in the guts of most of the antibiotic-treated groups. These changes were also associated with a significant decrease in Prevotella in both the AMP and VAN groups. Taken together, these findings indicate that changes in gut microbiota as well as host physiology, including host metabolism and immunity, differ depending on the types of antibiotics, and the antibiotic-induced gut microbiota alteration has a correlation with host physiology such as host metabolic or immunological status. Thus, the immune and metabolic status of the host should be taken into account when administering antibiotics.

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Disorders of phenylalanine and tyrosine metabolism

Cited by 14 publications

References 233 publications

Coconut Carbon Dots: Progressive Large-Scale Synthesis, Detailed Biological Activities and Smart Sensing Aptitudes towards Tyrosine

Coconut Carbon Dots: Progressive Large-Scale Synthesis, Detailed Biological Activities and Smart Sensing Aptitudes towards Tyrosine

Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria

Distinct Changes in Microbiota-Mediated Intestinal Metabolites and Immune Responses Induced by Different Antibiotics

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