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Related Concept Videos

Overview of Protein Metabolism01:21

Overview of Protein Metabolism

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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.
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Protein Kinases and Phosphatases02:54

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Proteins undergo chemical modifications that trigger changes in the charge, structure, and conformation of the proteins. Phosphorylation, acetylation, glycosylation, nitrosylation, ubiquitination, lipidation, methylation, and proteolysis are various protein modifications that regulate protein activity. Such modifications are usually enzyme-driven.
Protein kinases
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Protein Complexes with Interchangeable Parts01:57

Protein Complexes with Interchangeable Parts

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Groups of proteins may form a complex where each protein in this complex has a different role in the overall execution of the complex’s function. Often some of the proteins in the complex can be replaced by a closely related variant to give a complex that contains many of the same components yet is functionally distinct.
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Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

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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...
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Conservation of Protein Domains Over Different Proteins02:26

Conservation of Protein Domains Over Different Proteins

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Protein domains are small structurally independent units that are part of a single amino acid chain.  Although these domains are often structurally independent, they may rely on synergistic effects to perform their functions as part of a larger protein. Protein domains may be conserved within the same organism, as well as across different organisms.
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Protein Families02:47

Protein Families

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Protein families are groups of homologous proteins; that is, they have similarities in amino acid sequences and three-dimensional structures. Protein families usually occur because of gene duplication, where an additional copy of a gene is inserted into the genome of an organism.   Mutations that change the amino acids but still allow the protein to be properly synthesized, will lead to new protein family members.   If these new proteins contain similar amino acids in key...
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Real-World Administration Practices of Sapropterin in Paediatric and Adults with Phenylketonuria: Results from a United Kingdom Cross-Sectional Survey.

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Blood Phenylalanine Control in Paediatric and Adult Centres in the UK: Data from 2012-2018.

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How Well Is Blood Phenylalanine Controlled in Maternal PKU in Europe? Results from 102 Pregnancies.

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Identification of Novel CK2 Kinase Substrates Using a Versatile Biochemical Approach
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Protein Substitutes in PKU; Their Historical Evolution.

Anne Daly1, Sharon Evans1, Alex Pinto1

  • 1Birmingham Women's and Children's Hospital, NHS Foundation Trust, Birmingham B4 6NH, UK.

Nutrients
|February 5, 2021
PubMed
Summary

Protein substitutes are vital for managing phenylketonuria (PKU), providing essential amino acids. Advances in formulation, like bioactive macropeptides and slow-release technology, improve treatment efficacy and patient health.

Keywords:
amino acidglycomacropeptidephenylketonuriaprotein substitute

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Area of Science:

  • Biochemistry
  • Nutritional Science
  • Metabolic Disorders

Background:

  • Protein substitutes, typically L-amino acid-based, are crucial for managing phenylketonuria (PKU) and other amino acid disorders.
  • These formulations enable the removal of antagonistic amino acids while supplying essential ones for physiological function.
  • The development of PKU protein substitutes has evolved significantly since their initial experimental use.

Observation:

  • Since 2008, bioactive macropeptides have been utilized as a base for PKU protein substitutes.
  • Animal studies in 2018 demonstrated physiomimic technology for slow amino acid release, enhancing physiological profiles.
  • Efficacy of PKU protein substitutes depends on nutritional profile, amino acid composition, dosage, timing, and energy intake.

Findings:

  • Bioactive macropeptides offer potential benefits including improved bone and gut health, nitrogen retention, and better blood phenylalanine control.
  • Physiomimic technology facilitates a slow release of amino acids, leading to an improved physiological profile.
  • Optimizing nutritional profile, amino acid composition, dose, timing, distribution, and energy intake is key to protein substitute efficacy.

Implications:

  • Protein substitutes play a pivotal role in the pharmacological actions and clinical management of PKU.
  • Continued innovation in protein substitute formulation can lead to enhanced therapeutic outcomes for PKU patients.
  • Recognizing the importance of protein substitutes is essential for effective PKU management and improving patient quality of life.