Tyrosine and Phenylalanine Metabolism

Vitamin C ◽  
2018 ◽  
pp. 75-92
Author(s):  
C. Alan B. Clemetson
2021 ◽  
pp. 1-14
Author(s):  
Pan Liu ◽  
Qian Yang ◽  
Ning Yu ◽  
Yan Cao ◽  
Xue Wang ◽  
...  

Background: Alzheimer’s disease (AD) is one of the most challenging diseases causing an increasing burden worldwide. Although the neuropathologic diagnosis of AD has been established for many years, the metabolic changes in neuropathologic diagnosed AD samples have not been fully investigated. Objective: To elucidate the potential metabolism dysregulation in the postmortem human brain samples assessed by AD related pathological examination. Methods: We performed untargeted and targeted metabolomics in 44 postmortem human brain tissues. The metabolic differences in the hippocampus between AD group and control (NC) group were compared. Results: The results show that a pervasive metabolic dysregulation including phenylalanine metabolism, valine, leucine, and isoleucine biosynthesis, biotin metabolism, and purine metabolism are associated with AD pathology. Targeted metabolomics reveal that phenylalanine, phenylpyruvic acid, and N-acetyl-L-phenylalanine are upregulated in AD samples. In addition, the enzyme IL-4I1 catalyzing transformation from phenylalanine to phenylpyruvic acid is also upregulated in AD samples. Conclusion: There is a pervasive metabolic dysregulation in hippocampus with AD-related pathological changes. Our study suggests that the dysregulation of phenylalanine metabolism in hippocampus may be an important pathogenesis for AD pathology formation.


PEDIATRICS ◽  
1969 ◽  
Vol 44 (5) ◽  
pp. 655-660
Author(s):  
Bernard E. Cohen ◽  
Arieh Szeinberg ◽  
Wifred Berman ◽  
Yermiahu Aviad ◽  
Moshe Crispin ◽  
...  

A highly inbred family with five mentally retarded persons is described. Two sibs presented typical characteristics of phenylketonuria, while one mentally retarded sib did not show any biochemical abnormality. The mother and maternal uncle had mild hyperphenylalaninemia. It is pointed out in the discussion that, while the mental retardation (at least in some of these subjects) may be independent of disturbances of phenylalanine metabolism, it is possible also to explain all the findings in the family on a unified basis, involving a variant hyperphenylalaninemia with tolerance increasing with age and "maternal phenylketonuia."


Genetics ◽  
1966 ◽  
Vol 54 (6) ◽  
pp. 1391-1399
Author(s):  
V G Zannoni ◽  
W W Weber ◽  
P Van Valen ◽  
A Rubin ◽  
R Bernstein ◽  
...  

1986 ◽  
Vol 7 (9) ◽  
pp. 269-275
Author(s):  
Harvey L. Levy

Phenylketonuria (PKU) has been aptly described as the "epitome of human biochemical genetics." In so distinguishing PKU among the many metabolic disorders now known, Scriver and Clow identified several categories in which this inborn error of metabolism is singularly prominent. First and foremost, PKU represents a fusion of effort between public health and genetics. It is the major genetic disorder in which treatment can prevent the clinical expression of disease and for which routine biochemical screening of newborn infants was developed. It remains the model for such screening. Second, PKU is the prime example of the importance of understanding as completely as possible the biochemical basis of a metabolic disorder. The detailed understanding of phenylalanine metabolism that arose from studies spawned by PKU led to the recognition of "new" metabolic disorders that relate to PKU in their capacity to increase the phenylalanine level but that involve a different category of metabolism and require very different treatment. Third, PKU represents an important link between obstetrics and pediatrics. The threat to the fetus from PKU in the pregnant woman (maternal PKU) must be met by special dietary care throughout the pregnancy. This is, perhaps, only the first of other maternal inborn errors that will require similar intervention during pregnancy.


Author(s):  
Mary Ann Richardson ◽  
Cheryl Flynn ◽  
Laura Read ◽  
Margaret Reilly ◽  
Raymond Suckow

2020 ◽  
Vol 9 (10) ◽  
pp. 3248
Author(s):  
Patricia Kink ◽  
Eva Maria Egger ◽  
Lukas Lanser ◽  
Michaela Klaunzner ◽  
Bernhard Holzner ◽  
...  

Anemia often coincides with depression and impaired quality of life (QoL) in cancer patients. Sustained immune activation can lead to the development of anemia. Furthermore, it also may go along with changes in tryptophan and phenylalanine metabolism. The aim of our pilot study was to study the relationship between anemia, immune-mediated changes in amino acid metabolism, and the QoL and mood of cancer patients. Questionnaires to measure QoL and depression were completed by 152 patients with solid tumors. Hemoglobin, parameters of immune activation as well as tryptophan and phenylalanine metabolism were determined in the patients’ sera. Anemic patients (51.7%) presented with higher inflammatory markers, and a higher tryptophan breakdown with lower tryptophan concentrations. They reported an impaired QoL and had higher depression scores. Patients with an impaired QoL (65.8%) also suffered from more fatigue and impaired physical, emotional, and social functioning. They, furthermore, presented with higher concentrations of inflammatory markers (C-reactive protein (CRP) and neopterin) as well as higher tryptophan degradation (in men) and higher phenylalanine concentrations (in women). Sixty-one patients (40.1%) had (mostly mild) depression. In these patients, a higher degree of Th1 immune activation was found. The results of our study suggest that cancer-related anemia goes along with an impaired QoL, which is also associated with immune-mediated disturbances of tryptophan and phenylalanine metabolism.


Plants ◽  
2020 ◽  
Vol 9 (2) ◽  
pp. 204 ◽  
Author(s):  
Xu ◽  
Su ◽  
Zhao ◽  
Jin ◽  
Cheng ◽  
...  

Plant hormones play an important role in the chemical metabolism of postharvest plants. However, alterations in plant hormones of postharvest tea and their potential modulation of quality-related metabolites are unknown. In this study, the dynamic alterations of abscisic acid (ABA), salicylic acid (SA), jasmonic acid (JA), and critical metabolites, such as catechins, theanine, and caffeine, in tea leaves were analyzed during withering from 0 to 24 h. It was found that the ABA content increased from 0 to 9 h but decreased thereafter, JA continuously increased, and the SA content showed no significant change. With the exception of gallocatechin (GC) and epicatechin (EC), the amounts of other critical components were significantly reduced at 24 h. Transcriptome analysis showed that compared with 0 h, 2256, 3654, and 1275 differentially expressed genes (DEGs) were identified at 9, 15, and 24 h, respectively. For all comparisons, DEGs corresponding to the pathways of “phenylalanine, tyrosine, and tryptophan biosynthesis” and “phenylalanine metabolism”, involved in the biosynthesis of catechins, were significantly enriched. Weighted correlation network analysis (WGCNA) of co-expression genes indicated that many of the modules were only correlated with a specific trait during the withering process; the dark olive-green module, however, was correlated with two traits, ABA and theanine. Our study indicates that withering induced dramatic alterations in gene transcription as well as levels of hormones (ABA, JA, and SA) and important components, and that ABA regulated theanine metabolism during this process.


1962 ◽  
Vol 17 (6) ◽  
pp. 985-992 ◽  
Author(s):  
William Sacks

After injection of dl-phenylalanine-1-C14 or l-phenylalanine-1-C14, C14O2 specific activities in whole blood were frac15–frac13 as high as those after injection of dl-tyrosine-1-C14 or l-tyrosine-1-C14 in normal subjects and chronic psychotic patients. In phenylalanine-C14 studies, tyrosine specific activities were 1/16–1/10 of corresponding phenylalanine specific activities. After injection of l-phenylalanine-1-C14 into two phenylketonuric (PKU) patients, C14O2 activities in one, a child, approximated those in other experiments, whereas they were lower in the adult PKU-patient. Maximal tyrosine specific activity (adult PKU patient) was frac16 the corresponding phenylalanine specific activity. Ratios of specific activities of maximal C14O2 to phenylalanine were similar in the control subject and PKU patient, suggesting that catabolism of phenylalanine proceeds at the same rate in phenylketonuria. Results with l-phenylalanine-U-C14 indicated that more than the carboxyl carbon contributed to blood 14O2. The data suggest that hydroxylation of phenylalanine to form tyrosine may be a minor pathway in intermediary metabolism of phenylalanine in normal humans and in chronic psychotic and phenylketonuric patients. Submitted on April 11, 1962


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