Current Landscape on Development of Phenylalanine and Toxicity of its Metabolites - A Review

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Phenylalanine, an essential amino acid, is the "building block" of protein. It has a tremendous role in different aspects of metabolic events. The tyrosine pathway is the prime one and is typically used to degrade dietary phenylalanine. Phenylalanine exceeds its limit in bodily fluids and the brain when the enzyme, phenylalanine decarboxylase, phenylalanine transaminase, phenylalanine hydroxylase (PAH) or its cofactor tetrahydrobiopterin (BH4) is deficient causes phenylketonuria, schizophrenia, attentiondeficit/ hyperactivity disorder and another neuronal effect. Tyrosine, an amino acid necessary for synthesizing the pigments in melanin, is produced by its primary metabolic pathway. Deficiency/abnormality in metabolic enzymes responsible for the catabolism pathway of Phenylalanine causes an accumulation of the active intermediate metabolite, resulting in several abnormalities, such as developmental delay, tyrosinemias, alkaptonuria, albinism, hypotension and several other undesirable conditions. Dietary restriction of the amino acid(s) can be a therapeutic approach to avoid such undesirable conditions when the level of metabolic enzyme is unpredictable. After properly identifying the enzymatic level, specific pathophysiological conditions can be managed more efficiently.

Medienart:

E-Artikel

Erscheinungsjahr:

2024

Erschienen:

2024

Enthalten in:

Zur Gesamtaufnahme - volume:19

Enthalten in:

Current drug safety - 19(2024), 2 vom: 27., Seite 208-217

Sprache:

Englisch

Beteiligte Personen:

Bose, Samrat [VerfasserIn]
Mandal, Shirsendu [VerfasserIn]
Khan, Rajesh [VerfasserIn]
Maji, Himangshu Sekhar [VerfasserIn]
Ashique, Sumel [VerfasserIn]

Links:

Volltext

Themen:

42HK56048U
47E5O17Y3R
Amino Acids
Congenital abnormalities
Dietary restriction.
EC 1.14.16.1
Journal Article
Metabolic pathways
Phenylalanine
Phenylalanine Hydroxylase
Phenylketonuria
Tyrosine
Tyrosinemias

Anmerkungen:

Date Completed 10.01.2024

Date Revised 10.01.2024

published: Print

Citation Status MEDLINE

doi:

10.2174/1574886318666230331112800

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM355046199