Tipo
Capítulo em Livro
Título
CHAPTER 37. Riboflavin and Beta-oxidation Flavoenzymes
Participantes na publicação
Bárbara J. Henriques (Author)
Dep. Química e Bioquímica
BioISI
João V. Rodrigues (Author)
Cláudio M. Gomes (Author)
Resumo
Riboflavin, or vitamin B2, is the biological precursor of the essential redox cofactors FAD and FMN, which are synthesised upon dietary intake of the vitamin. The unique physical and chemical properties of these flavins make them extremely versatile protein cofactors. When inserted into proteins, flavins afford a broad range of redox reactions and catalytic properties. In this chapter we focus on the interplay between flavoproteins involved in mitochondrial beta-oxidation and vitamin B2 metabolism, especially in what concerns the protein functional and structural consequences of increased flavin levels in this organelle, subsequently to high-dose riboflavin intake. The latter aspect is particularly relevant in the context of inborn errors of metabolism affecting beta-oxidation and amino acid catabolism enzymes, frequently a consequence of missense mutations, which result in protein misfolding or catalytic impairment. We provide an overview of recent reports establishing that therapeutic intake of vitamin B2 increases cellular flavin concentrations, thus modulating the levels of several proteins involved on this pathway by rescuing function, and we discuss the molecular and structural rationale for these effects. Ultimately, we describe a mechanistic scenario pinpointing the role exerted by flavins as protein folding helpers.
Editor
Victor R Preedy
Data de Publicação
2012-10-23
Instituição
INSTITUTO DE TECNOLOGIA QUÍMICA E BIOLÓGICA
Suporte
B Vitamins and Folate,Food and Nutritional Components in Focus
Identificadores da Publicação
ISSN - 2045-1709
ISBN - 9781849733694
Editora
Royal Society of Chemistry
Número de Páginas
21
Página Inicial
611
Página Final
632
Identificadores do Documento
DOI -
https://doi.org/10.1039/9781849734714-00611
URL -
http://dx.doi.org/10.1039/9781849734714-00611