Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

530
Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
530
Amino Acid Biosynthetic Pathways01:29

Amino Acid Biosynthetic Pathways

526
Amino acid biosynthesis is essential for cell growth, protein synthesis, and metabolic regulation. Cells generate essential and non-essential amino acids from metabolic intermediates to sustain vital biological functions. These intermediates originate from key metabolic pathways: glycolysis, the tricarboxylic acid (TCA) cycle, and the pentose phosphate pathway. Important precursors include α-ketoglutarate, pyruvate, oxaloacetate, phosphoenolpyruvate, and erythrose-4-phosphate, which...
526
Overview of Protein Metabolism01:21

Overview of Protein Metabolism

2.9K
Proteins are broken down into amino acids during digestion. Unlike fats and carbohydrates, which are stored for later use, proteins are not. Instead, amino acids are either used to produce ATP through oxidation or contribute to the creation of new proteins for the growth and repair of the body. Any surplus amino acids from the diet are converted into glucose or triglycerides rather than excreted.
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...
2.9K
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

89
In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
89
Allosteric Proteins-ATCase01:19

Allosteric Proteins-ATCase

6.2K
Binding sites linkages can regulate a protein's function.  For example, enzyme activity is often regulated through a feedback mechanism where the end product of the biochemical process serves as an inhibitor.
Aspartate transcarbamoylase (ATCase) is a cytosolic enzyme that catalyzes the condensation of L-aspartate and carbamoyl phosphate to  N-carbamoyl-L-aspartate. This reaction is the first step in pyrimidine biosynthesis. UTP and CTP, the end products of the pyrimidine synthesis...
6.2K
Chronic Kidney Disease III: Interprofessional Care01:28

Chronic Kidney Disease III: Interprofessional Care

206
Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...
206

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

CFTR modulator monotherapy for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).

The Cochrane database of systematic reviews·2026
Same author

Lung transplant in people with cystic fibrosis and nontuberculous mycobacteria infection.

The Cochrane database of systematic reviews·2026
Same author

Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis.

The Cochrane database of systematic reviews·2025
Same author

Antibiotic treatment for non-tuberculous mycobacteria lung infection in people with cystic fibrosis.

The Cochrane database of systematic reviews·2025
Same author

Intravenous antibiotics for pulmonary exacerbations in people with cystic fibrosis.

The Cochrane database of systematic reviews·2025
Same author

Infection prevention and control in cystic fibrosis: An update of a systematic review of interventions.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society·2024

Related Experiment Video

Updated: Nov 22, 2025

Simultaneous Quantification of Selected Kynurenines Analyzed by Liquid Chromatography-Mass Spectrometry in Medium Collected from Cancer Cell Cultures
10:38

Simultaneous Quantification of Selected Kynurenines Analyzed by Liquid Chromatography-Mass Spectrometry in Medium Collected from Cancer Cell Cultures

Published on: May 9, 2020

3.6K

Tyrosine supplementation for phenylketonuria.

Tracey Remmington1, Sherie Smith2

  • 1Department of Women's and Children's Health, University of Liverpool, Liverpool, UK.

The Cochrane Database of Systematic Reviews
|January 11, 2021
PubMed
Summary

Tyrosine supplementation in phenylketonuria (PKU) significantly increased blood tyrosine levels but did not improve other outcomes. More research is needed, but this is not an active area of study.

More Related Videos

Liquid Chromatography Coupled to Refractive Index or Mass Spectrometric Detection for Metabolite Profiling in Lysate-based Cell-free Systems
14:42

Liquid Chromatography Coupled to Refractive Index or Mass Spectrometric Detection for Metabolite Profiling in Lysate-based Cell-free Systems

Published on: September 23, 2021

5.4K
Functional Complementation Analysis FCA: A Laboratory Exercise Designed and Implemented to Supplement the Teaching of Biochemical Pathways
09:27

Functional Complementation Analysis FCA: A Laboratory Exercise Designed and Implemented to Supplement the Teaching of Biochemical Pathways

Published on: June 24, 2016

18.0K

Related Experiment Videos

Last Updated: Nov 22, 2025

Simultaneous Quantification of Selected Kynurenines Analyzed by Liquid Chromatography-Mass Spectrometry in Medium Collected from Cancer Cell Cultures
10:38

Simultaneous Quantification of Selected Kynurenines Analyzed by Liquid Chromatography-Mass Spectrometry in Medium Collected from Cancer Cell Cultures

Published on: May 9, 2020

3.6K
Liquid Chromatography Coupled to Refractive Index or Mass Spectrometric Detection for Metabolite Profiling in Lysate-based Cell-free Systems
14:42

Liquid Chromatography Coupled to Refractive Index or Mass Spectrometric Detection for Metabolite Profiling in Lysate-based Cell-free Systems

Published on: September 23, 2021

5.4K
Functional Complementation Analysis FCA: A Laboratory Exercise Designed and Implemented to Supplement the Teaching of Biochemical Pathways
09:27

Functional Complementation Analysis FCA: A Laboratory Exercise Designed and Implemented to Supplement the Teaching of Biochemical Pathways

Published on: June 24, 2016

18.0K

Area of Science:

  • Metabolic Disorders
  • Genetics
  • Nutritional Science

Background:

  • Phenylketonuria (PKU) is an inherited disorder requiring strict phenylalanine restriction.
  • The restrictive PKU diet can be challenging and may lead to tyrosine deficiency.
  • Tyrosine deficiency is implicated in neuropsychological issues observed in PKU patients.

Purpose of the Study:

  • To evaluate the efficacy of tyrosine supplementation in PKU patients.
  • To assess tyrosine's impact on intelligence, neuropsychological performance, growth, and quality of life.
  • To determine if tyrosine supplementation is beneficial alongside or instead of a phenylalanine-restricted diet.

Main Methods:

  • Systematic review of randomized and quasi-randomized controlled trials.
  • Searched multiple databases and contacted manufacturers for relevant studies.
  • Included trials involving tyrosine supplementation versus placebo in PKU patients.

Main Results:

  • Three trials with 56 participants were included.
  • Tyrosine supplementation significantly increased blood tyrosine concentrations.
  • No significant differences were observed in other measured outcomes like intelligence or growth.

Conclusions:

  • Current evidence does not support routine clinical use of tyrosine supplementation for PKU.
  • Further randomized controlled studies are necessary to confirm benefits.
  • This review will not be updated due to limited research activity in this area.