IMR Press / FBE / Volume 4 / Issue 3 / DOI: 10.2741/E428

Frontiers in Bioscience-Elite (FBE) is published by IMR Press from Volume 13 Issue 2 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with Frontiers in Bioscience.

Open Access Review

Biomedical aspects of pyridoxal 5’-phosphate availability

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1 Dipartimento di Scienze Biochimiche, A. Rossi Fanelli and Istituto Pasteur-Fondazione Cenci Bolognetti, Sapienza Universita di Roma, Piazzale Aldo Moro 5, 00185, Roma, Italy
2 Department of Medicinal Chemistry, School of Pharmacy, and Institute for Structural Biology and Drug Discovery, Virginia Commonwealth University, Richmond, Virginia 23219, USA

*Author to whom correspondence should be addressed.

Academic Editor: Carla Borri Voltattorni

Front. Biosci. (Elite Ed) 2012, 4(3), 897–913; https://doi.org/10.2741/E428
Published: 1 January 2012
(This article belongs to the Special Issue Biomedical aspects of pyridoxal phosphate dependent enzymes)
Abstract

The biologically active form of vitamin B6, pyridoxal 5’-phosphate (PLP), is a cofactor in over 160 enzyme activities involved in a number of metabolic pathways, including neurotransmitter synthesis and degradation. In humans, PLP is recycled from food and from degraded PLP-dependent enzymes in a salvage pathway requiring the action of pyridoxal kinase, pyridoxine 5’-phosphate oxidase and phosphatases. Once pyridoxal 5’-phosphate is made, it is targeted to the dozens different apoenzymes that need it as a cofactor. The regulation of the salvage pathway and the mechanism of addition of PLP to the apoenzymes are poorly understood and represent a very challenging research field. Severe neurological disorders, such as convulsions and epileptic encephalopathy, result from a reduced availability of pyridoxal 5’-phosphate in the cell, due to inborn errors in the enzymes of the salvage pathway or other metabolisms and to interactions of drugs with PLP or pyridoxal kinase. Multifactorial neurological pathologies, such as autism, schizophrenia, Alzheimer's disease, Parkinson’s disease and epilepsy have also been correlated to inadequate intracellular levels of PLP.

Keywords
Vitamin B6
Pyridoxal 5’-Phosphate
Salvage Pathway
Pyridoxal Kinase
Pyridoxine 5’-Phosphate Oxidase
Plp Deficiency
Neurological Disorders
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